Objective:Factors influencing the prognosis of hemophagocytic lymphohistiocytosis (HLH) in adults were analyzed based on multicentric data.Methods:Clinical data of 124 adult patients with HLH diagnosed in eight medical centers in the Huaihai Lymphoma Working Group from March 2014 to July 2020 were collected. The optimal truncation value of continuous variables was obtained based on the Maxstat algorithm, X-Tile software, and restricted cubic spline. Cox proportional risk regression model was used to construct the adult HLH risk prediction model, and the visualization of the model was realized through the histogram. The bootstrap resampling method was used to verify the model, C-index and calibration curve was used to verify the histogram, and the prediction accuracy was checked. Kaplan-Meier analysis was used to calculate the survival rate and draw the survival curve. Furthermore, the differences between groups were tested by log-rank.Results:The median age of the 124 patients was 55 (18-84) years, including 61 (49.19%) males. The most common etiology was infection. Serum ferritin increased in 110 cases (88.71%) , hepatosplenomegaly in 57 cases (45.97%) . Of the 124 patients, 77 (62.10%) died, and the median survival time of the patients was 7.07 months. Univariate results showed that the prognosis of adult HLH was influenced by sex, age, fibrinogen, serum creatinine, alanine aminotransferase, and albumin ( P<0.05) . The results of multivariate analysis showed that gender, platelet, albumin, alanine aminotransferase, and treatment regimens were independent influencing factors for prognosis. Based on the above five risk factors, the prediction model of the histogram was established, and the C-index of the model was 0.739. Finally, the calibration chart showed good consistency between the observed and predicted values of HLH. Conclusion:The prognosis of the adult hemophagocytic syndrome is influenced by many factors. Gender, platelet, albumin, alanine aminotransferase, and treatment regimens are independent risk factors. Therefore, the established histogram provides a visual tool for clinicians to evaluate the prognosis of adult HLH.

Objective: To explore hepatitis B virus (HBV) infection rate of breast feeding to newborn babies of HBV carrying parturient women with hepatitis B surface antigen (HBsAg) and hepatitis B e antigen (HBeAg) double positive. Methods: A prospective cohort study was conducted to include HBsAg and HBeAg double-positive HBV carrying parturient women and their babies born from February 2016 to May 2018 at the Women′s Hospital, Zhejiang University School of Medicine, and 323 parturient women and 323 babies were enrolled. The babies were divided into breast feeding group and artificial feeding group. Chemiluminescence immunoassay and polymerase chain reaction-fluorescent probe method were used to detect the positive rates of serum HBV markers and HBV DNA levels in the newborns <24 h and seven-month-old age, respectively. The statistical method was performed using χ² test. Results: A total of 297 parturient women were finally included for the analysis, including 149 in the breast feeding group and 148 in the artificial feeding group. There were no significant differences in the positive rates of HBsAg, hepatitis B surface antibody (anti-HBs), HBeAg and HBV DNA>100 IU/mL between the two groups at birth <24 h and seven months of age (all P>0.05). The positive rate of anti-HBs in newborns in the breast feeding group at birth <24 h was 58.39%(87/149), which was lower than 95.97%(143/149) at seven months of age. The HBeAg-positive group was 65.10%(97/149) at birth <24 h in the breast feeding group, which was higher than 13.42%(20/149) at seven months of age. The differences were both statistically significant (χ²=59.75 and 40.49, respectively, both P<0.01). The positive rates of HBsAg and HBV DNA>100 IU/mL in newborns in the breast feeding group were 2.01%(3/149) and 2.68%(4/149) at birth <24 h, respectively, and those at seven months were 2.68%(4/149) and 2.68%(4/149), respectively. There were no significant differences between the two time points (both P>0.05). In the artificial feeding group, the positive rate of anti-HBs in newborns was 47.97%(71/148) at birth <24 h, which was lower than 95.94% (142/148) at seven months of age. The positive rate of HBeAg in the artificial feeding group was 55.41%(82/148) in newborns at birth <24 h, which was higher than 19.59%(29/148) at seven months of age. The differences were statistically significant (χ²=85.37 and 39.84, respectively, both P<0.01). The positive rates of HBsAg and HBV DNA>100 IU/mL in newborns in the artificial feeding group at birth <24 h were 4.73%(7/148) and 1.35%(2/148), respectively, and those at seven months were 1.35%(2/148) and 1.35%(2/148), respectively. There were no significant differences between the two time points (both P>0.05). Conclusions Breast feeding is not a decisive factor for the risk of vertical transmission in HBsAg and HBeAg double-positive HBV carriers. It is recommended that such women could breastfeed under formal precautions.

Objective: To investigate the efficacy and safety of rapamycin in children with tuberous sclerosis complex (TSC) associated renal disease. Methods: A prospective self-control study was conducted. The clinical data of 92 children diagnosed with tuberous sclerosis complex associated kidney disease at the People′s Liberation Army General Hospital from January 2011 to January 2019 were collected. The long-term rapamycin treatment for all patients initiated at 1 mg/(m²·d), which was gradually adjusted to reach a blood concentration of 5-10 μg/L. The changes of the maximum diameter of renal lesions in children after rapamycin treatment were observed and analyzed with Wilcoxon test. Results: Ninety-two children, including 52 males and 40 females, who met the criteria were analyzed. Sixty patients had only renal angiomyolipoma(RAML), while 24 patients had only multiple renal cysts(MRC), and 8 patients had both lesions. The age of TSC diagnosis was 16.0 (7.0, 42.0) months, and the age of initial treatment with rapamycin was 63.5 (21.0, 103.0) months. The follow-up lasted for 12.0 (4.0, 23.0) months. Sequencing of TSC1 and TSC2 genes was performed in 54 children with TSC, including 3 patients (6%) with mutations in TSC1 gene and 51 patients (94%) with mutations in TSC2 gene. The maximum RAML diameter before treatment was 7.0 (4.0, 9.0) mm. The best effect reached at 3 months of treatment, with the diameter of 4.0 (0,7.0) mm. The maximum diameters at 6 months, 1 year and 1-2 years were 5.0 (0,9.8) mm, 5.0 (1.5, 8.5) mm, 5.5 (3.0, 9.0) mm, respectively, and were significantly different from the baseline (Z=-2.404,-2.350,-2.750,P=0.016,0.019,0.006, respectively). The maximum diameter after 2-3 years, and ≥3 years were 5.0 (3.9,7.0) mm and 6.0 (1.0, 11.0) mm, without significant difference from the baseline (Z=-0.856,-0.102,P=0.393,0.919, respectively).The maximum diameters of MRC after 3 months, 6 months, 1 year,1-2 years, 2-3 years, and ≥3 years were 11.0 (5.0, 14.0) mm,3.0 (0.0,11.0) mm,5.0 (0,21.0) mm,0 (0,14.0) mm,0 (0,10.0) mm, and 0 (0,18.3) mm, respectively, but were not significantly different rom the baseline (7.0 (5.0, 15.7) mm)(Z=-0.944,-1.214,-1.035,-1.896,-1.603,-1.214,P=0.345,0.225,0.301,0.058,0.109,0.225, respectively).Twenty-nine patients (32%) had oral ulcers during the entire treatment period, and no serious adverse reactions were observed. Conclusions Rapamycin could decrease the diameter of TSC-related RAML, but could not inhibit the growth of cysts. It is well tolerated in the treatment of renal diseases associated with tuberous sclerosis complex.

Objective To design a portable electroencephalography(EEG) acquisition system to acquire and analysis steady-state visual potentials (SSVEP). Methods The microprocessor MSP432P401 series MCU was used to control the high-performance integrated analog front end ADS1299 to realize the acquisition, amplification and analog-to-digital (AD) conversion of EEG signals. The digital EEG signal is sent to the host computer for processing by WIFI. Spontaneous EEG signals and steady-state visually evoked EEG signals from 3 healthy subjects were collected to verify system performance. Results The collected signal had a clear α-wave rhythm of closed-eye spontaneous EEG signals. The power spectrum density shows that the steady-state visually induced EEG signal frequency and harmonic frequency peak at the corresponding stimulation frequency, indicating that the system works normally and the performance is good. Conclusions The designed portable EEG acquisition system can accurately collect the spontaneous and induced EEG signals of the human body, which provides technical support for the clinical application of SSVEP technology.

Mood disorders/psychosis have been associated with dysfunctions in the default mode network (DMN). However, the relative contributions of DMN regions to state and trait disturbances in pediatric bipolar disorder (PBD) remain unclear. The aim of this study was to investigate the possible mechanisms of PBD through brain imaging and explore the influence of psychotic symptoms on functional alterations in PBD patients. Twenty-nine psychotic and 26 non-psychotic PBD patients, as well as 19 age- and sex-matched healthy controls underwent a resting-state functional MRI scan and the data were analyzed by independent component analysis. The DMN component from the fMRI data was extracted for each participant. Spearman's rank correlation analysis was performed between aberrant connectivity and clinical measurements. The results demonstrated that psychotic PBD was characterized by aberrant DMN connectivity in the anterior cingulate cortex/medial prefrontal cortex, bilateral caudate nucleus, bilateral angular gyri, and left middle temporal gyrus, while non-psychotic PBD was not, suggesting further impairment with the development of psychosis. In summary, we demonstrated unique impairment in DMN functional connectivity in the psychotic PBD group. These specific neuroanatomical abnormalities may shed light on the underlying pathophysiology and presentation of PBD.

To investigate the efficacy and safety of rapamycin in children with tuberous sclerosis complex (TSC) associated renal disease. Methods A prospective self?control study was conducted. The clinical data of 92 children diagnosed with tuberous sclerosis complex associated kidney disease at the People's Liberation Army General Hospital from January 2011 to January 2019 were collected. The long?term rapamycin treatment for all patients initiated at 1 mg/(m2·d), which was gradually adjusted to reach a blood concentration of 5-10 μg/L. The changes of the maximum diameter of renal lesions in children after rapamycin treatment were observed and analyzed with Wilcoxon test. Results Ninety?two children, including 52 males and 40 females, who met the criteria were analyzed. Sixty patients had only renal angiomyolipoma(RAML), while 24 patients had only multiple renal cysts(MRC), and 8 patients had both lesions. The age of TSC diagnosis was 16.0 (7.0, 42.0) months, and the age of initial treatment with rapamycin was 63.5 (21.0, 103.0) months. The follow?up lasted for 12.0 (4.0, 23.0) months. Sequencing of TSC1 and TSC2 genes was performed in 54 children with TSC, including 3 patients (6%) with mutations in TSC1 gene and 51 patients (94%) with mutations in TSC2 gene. The maximum RAML diameter before treatment was 7.0 (4.0, 9.0) mm. The best effect reached at 3 months of treatment, with the diameter of 4.0 (0, 7.0) mm. The maximum diameters at 6 months, 1 year and 1-2 years were 5.0 (0,9.8) mm, 5.0 (1.5, 8.5) mm, 5.5 (3.0, 9.0) mm, respectively, and were significantly different from the baseline (Z=-2.404,-2.350,-2.750, P=0.016,0.019,0.006, respectively). The maximum diameter after 2-3 years, and≥3 years were 5.0 (3.9,7.0) mm and 6.0 (1.0, 11.0) mm, without significant difference from the baseline (Z=-0.856,-0.102, P=0.393, 0.919, respectively).The maximum diameters of MRC after 3 months, 6 months, 1 year,1-2 years, 2-3 years, and≥3 years were 11.0 (5.0, 14.0) mm,3.0 (0.0,11.0) mm,5.0 (0,21.0) mm,0 (0,14.0) mm,0 (0,10.0) mm, and 0 (0, 18.3) mm, respectively, but were not significantly different rom the baseline (7.0 (5.0, 15.7) mm) (Z=-0.944,-1.214,-1.035,-1.896,-1.603,-1.214, P=0.345, 0.225, 0.301, 0.058, 0.109, 0.225, respectively). Twenty?nine patients (32%) had oral ulcers during the entire treatment period, and no serious adverse reactions were observed. Conclusions Rapamycin could decrease the diameter of TSC?related RAML, but could not inhibit the growth of cysts. It is well tolerated in the treatment of renal diseases associated with tuberous sclerosis complex.

Objective To study the effects of binary cardiac rehabilitation (CR) composed of hospital-based and home-based CR in patients after acute myocardial infarction (AMI) evaluated by six-minute walking distance (6MWD). Methods A total of 38 post-MI patients were divided into two groups, namely conventional CR group (n=20) and binary CR group (n=18). In the first seven days, CR training was carried out in all 38 patients in our hospital, and then 20 patients were kept at CR training in the hospital (hospital-based CR group), whereas the remaining 18 patients (binary CR group) got tele-monitored walking training at home for eight weeks (binary model). Before discharge from hospital based CR or after 8 weeks CR at home, all patients received Cardiopulmonary Exercise Testing and Six-minute Walk Test, and the data of patients' body mass index (BMI),thyroid function, serum lipid metabolism and echocardiography were collected before and after CR. Results After CR, BMI and low density lipoprotein (LDL) decrease in both groups compared with those before CR[hospital-based group: BMI, (25.99±3.36)kg/m2 vs. (23.04±3.72) kg/m2,P＜0.05; LDL, (3.40±1.38) mg/dl vs. (2.04±0.73)mg/ dl,P＜0.01; binary group: BMI, (24.84±2.70) kg/m2 vs. (22.88±2.56) kg/m2,P＜0.05; LDL, (3.40±1.01) mg/dl vs. (92.11±0.37) mg/dl,P＜0.01]. After 8 weeks CR at home or hospital based CR until discharge, the anaerobic threshold (AT), maximum rate of oxygen consumption (VO2max),metabolic equivalent (MET) and 6MWD improved significantly in both groups compared with those at 7 days after CR in hospital [Hospital-based group: AT, (12.37±1.53) mL/(kg. min) vs. (14.77±1.57) mL/(kg. min); VO2max (17.87± 1.66 mL/(kg. min) vs. (20.73±2.14) mL/(kg. min); MET (5.02±0.36) vs. (6.09±0.53); 6MWD (500±53.36) m vs. (582.5±57.6) m,P＜0.01; Binary group: AT, (12.56±1.11) mL/(kg·min) vs. (14.30±1.23) mL/(kg. min); V02max, (17.28±1.38) mL/(kg. min) vs. (20.02±1.37) mL/(kg. min); MET, (5.07±0.47)vs (5.94±0.46); 6MWD,(511.4±50.96) m vs. (590.3±56.1)m,P＜0.01]. There was no significant difference in CR effects observed between two groups (P＞0.05). Conclusions In post-MI patients,a binary model of CR training improved physical capacity and was a similarly effective form of CR as a entirely hospital-based approach. A home-based tele-monitored program facilitated patients' adherence to CR. The 6 minute walk experiment is economical and good evaluation on the CR effect of binary cardiac rehabilitation.

Objective: To explore the effectiveness of Balint group on compassion fatigue among oncology nurses. Methods: From January to December 2016, 35 oncology nurses from one general hospital were enrolled. 18 cases were allocated in the observation group and 17 cases in the control group by computer randomization. Nurses in the observation group were received a total of 8 times Balint Group activities, with 2 times a month and 1.5 hours each time, which is aiming to discuss difficult cases encountered in the clinic to help nurses have a deeper experience and a better understanding of the emotions and behaviors, fantasies and needs between nurse-patient interaction. Nurses in the control group without intervention. All the Participants were requested to complete the survey of the Professional Quality of Life (ProQOL) , the Jefferson Scale of Empathy-Health Professionals (JSE-HP) , and the General Health Questionnaire (GHQ) at pre and post intervention. Results: Before intervention, two group of nurses in age, working years, marriage, and education were not statistically significant (P>0.05). There was no difference in the scores of empathy, compassion satisfaction, secondary traumatic stress, and General health (P>0.05). After intervention, the scores of JSE-HP and its three dimensions of perspective taking, emotional care, and trans-positional consideration in the observation group had significantly higher than the control group (P<0.05). The level of compassion satisfaction in the observation group was higher, and the general health status was lower (P<0.05). There was no statistically difference in the level of burnout and secondary traumatic stress between two groups (P>0.05) . Conclusion Balint group has a positive role in promoting nurses’empathetic skills, compassion satisfaction and mental health.

Objective To analyze the clinical characteristics of Segawa syndrome to strengthen the understanding of Segawa syndrome and its differential diagnosis with Parkinson's disease.Methods The clinical data of a patient with Segawa syndrome and the family members were retrospectively analyzed.After informed consent,the peripheral blood of the patient and her patients were collected,and DNAs were extracted;high throughput sequencing was used to detect the hereditary movement disorders related genes and Sanger sequencing was performed to verify the results.Results Many patients in this family had onset at adult,mainly presented parkinsonism and dystonia,which was misdiagnosed as having Parkinson's disease.The proband was child onset and responded favorably to small dose of dopamine,and DNA sequencing showed a mutation of Guanosine triphosphate cyclohydrolase-1 gene,diagnosing as having Segawa syndrome.The same mutation existed in the father ofproband and not in her mother.Conclusion It is easy to confuse the adult Segawa syndrome with Parkinson's disease,and gene detection is an effective differential diagnosis method.

Objective To investigate the diagnostic value of blood homocysteine,ankle -brachial index and brachial -ankle pulse wave velocity in elderly patients with coronary heart disease (CHD).Methods 97 patients with routine coronary angiography were classified into CHD group (65 cases)and non -CHD group (32 cases) according to the results of coronary angiography.There were 24 cases with single -vessel disease in 65 CHD cases, 21 cases with double -vessel disease and 20 patients with multivessel disease of CHD.Basic clinical parameters,age, gender,TC,TG,LDL -C,HDL -C,etc and blood HCY,ABI,baPWV levels were compared among groups.Results The age of double -vessel disease group,multivessel disease group was significantly higher than that in single -vessel disease group(t =3.721,3.927,all P <0.05).HCY,ABI,baPWV in CHD group were (18.29 ±2.73)μmol/L, (0.97 ±0.16),(16.38 ±1.27)m/s,which had statistically significant differences compared with non -CHD group (HCY:t =5.701,P <0.01;ABI:t =6.138,P <0.01;baPWV:t =15.132,P <0.01 ).There were no significant differences between single -vessel disease group and non -CHD group on the ABI(all P >0.05),and the ABI of multi -vessel disease,double vessel disease group were significantly lower than that of non -CHD group (all P <0.01).HCY,baPWV of CHD group were significantly higher than non -CHD group(all P <0.01 ),double vessel disease,HCY multivessel disease group,ABI,baPWV average water with single -vessel disease group were signifi-cantly different(all P <0.01 ).With the increase of coronary lesions involved,the blood HCY,baPWV showed an increasing trend and ABI showed an decreasing trend.Conclusion Combined detection of HCY,baPWV and ABI has great value in early detection and early intervention of CHD in the elderly.