Objective:To investigate the clinical characteristics, muscle pathological features and treatment in patients with Juvenile idiopathic inflammatory myopathy (JIIM) with positive anti-nuclear matrix protein 2 (NXP2) antibody.Methods:The clinical data of 8 IMM patients with positive anti-NXP2 antibody were collected and the clinical manifestations, auxiliary examinations, muscle pathological changes and therapeutic effects were retrospectively analyzed.Results:The ratio of male to female was 1:3. The median age of disease onset was (6.1±3.8) years. Eight cases had proximal muscle weakness, 7 had dermatomyositis-like rash, 5 had myalgia,4 had calcinosis,3 had skin ulcer, 2 had edema and 1 had abdominal pain. Five cases had elevated serum creatine kinase. Eight cases with lower limb muscle MRI showed abnormal signals in muscle, space between muscles and fat tissue, 3 cases with chest high-resolution CT (HRCT) showed interstitial lung disease. Abdominal CT of 1 case showed irregular thickening, edema and peripheral inflammatory exudation in ascending colon and proximal transverse colon. Pathological biopsy of skeletal muscle showed perifascicular atrophy, inflammatory cell infiltration in fascicular membrane and around small vessels and muscle fiber space. Edema, hyperplasia could be seen in interstitium; but dissolved necrosis, and regenerated muscle fibers were rarely seen. Treatments included glucocorticoids, immunosuppressive agents and biological agents (1 case). After 6 months of follow-up, 5 cases had good outcomes and 3 cases had poor outcomes.Conclusion:Dermatomyositis is the major clinical manifestation of idiopathic inflammatory myopathy with positive anti-NXP2 antibody.It is associated with myasthenia, calcinosis, skin ulcers and intestinal vasculitis. The pathological changes in skeletal muscle are relatively slightmild. Glucocorticoids combined with immunosuppressive agents are effective in most cases.

Objective:To analyze the difference of clinical characteristics and outcomes of infants with moderate and severe pediatric acute respiratory distress syndrome(PARDS)diagnosed according to baseline oxygenation index(OI) in pediatric intensive care unit(PICU).Methods:Second analysis of the data collected from the "Efficacy of pulmonary surfactant (PS) in the treatment of children with moderate and severe ARDS" program.Retrospectively compare of the differences in clinical data such as general condition, underlying diseases, OI, mechanical ventilation, PS administration and outcomes among infants with moderate and severe PARDS divided by baseline OI who admitted to PICUs at 14 participating tertiary hospitals from 2016 to December 2021.Results:Among the 101 cases, 55 cases (54.5%) were moderate and 46 cases (45.5%) were severe PARDS.The proportion of male in the severe group (50.0% vs.72.7%, P=0.019) and the pediatric critical illness score(PCIS)[72 (68, 78) vs.76 (70, 80), P=0.019] were significantly lower than those in the moderate group, while there was no significant difference regarding age, body weight, etiology of PARDS and underlying diseases.The utilization rate of high-frequency ventilator in the severe group was significantly higher than that in the moderate group (34.8% vs.10.9%, P=0.004), but there was no significant difference in PS use, fluid load and pulmonary complications.The 24 h OI improvement (0.26±0.33 vs.0.04±0.34, P=0.001) and the 72 h OI improvement[0.34 (-0.04, 0.62) vs.0.15 (-0.14, 0.42), P=0.029)]in the severe group were significantly better than those in the moderate group, but there was no significant difference regarding mortality, length of hospital stay and intubation duration after diagnosis of PARDS between the two groups. Conclusion:In moderate and severe(divided by baseline OI) PARDS infants with invasive mechanical ventilation, children in severe group have better oxygenation improvement in the early stage after PARDS identified and are more likely to receive high frequency ventilation compared to those in moderate group.Baseline OI can not sensitively distinguish the outcomes and is not an ideal index for PARDS grading of this kind of patient.

Objective:To investigate the clinical characteristics and prognosis of children with septic shock caused by invasive pneumococcal diseases (IPDs) in pediatric intensive care unit (PICU).Methods:The clinical data of children diagnosed as septic shock caused by IPDs and hospitalized in the intensive care unit (ICU) of Beijing Children′s Hospital, Capital Medical University and the PICU of Henan Children′s Hospital from January 2013 to August 2019 were retrospectively collected, and the clinical characteristics and prognosis of these patients were analyzed.Results:Twenty-one children were included, with a median age of 1.2 (0.75, 3.90) years old.The pediatric index of mortality 2 (PIM-2) at admitting was (23.3±29.6)%, and 6 cases had underlying diseases.Main sites of infection included blood flow (20 cases) and suppurative meningitis (15 cases). The drug sensitivity test was performed on 18 children, among who 9 cases were sensitive to Penicillin, 10 cases to Cefepime, 11 cases to Cefotaxime and 10 cases to Meropenem.All 18 patients were sensitive to Vancomycin and Linezolid.Seven cases and 13 cases were treated with sensitive antibiotics at the disease onset and before septic shock, respectively.In 21 cases whose lactic acid level was (6.1±4.6) mmol/L, the shock redress time of 10 cases was (10.9±10.1)h, and 13 cases (61.9%) died (14.6±12.2) hours after septic shock, among who 10 died of transforamed magna herniation.The PIM-2 score at admitting into PICU and the rate of intracranial hypertension crisis in the death group were significantly higher than those in the survival group [(37.1±30.3)% vs.(0.9±1.3)%, 69.9% (9/13 cases) vs.25.0% (2/8 cases)](all P<0.05). There was no significant difference in age and the utilization rate of effective antibiotics before septic shock between the two groups (all P<0.05). Four of the surviving 8 children had severe cerebral functional disability. Conclusions:Septic shock caused by IPD is more common in children under 5 years old, and the most common sites are blood flow and intracranial infection.It has high resistance rate against Cephalosporins and Carbopenem.Patients with purulent meningitis are easy to develop intracranial hypertension crisis, which has an extremely high mortality and morbidity, so it needs to be identified and treated early.

Objective:To evaluate the efficacy and safety of nasal continuous positive airway pressure(NCPAP) in the treatment of congenital airway stenosis with severe pneumonia.Methods:A single-center retrospective clinical study was used to select children with congenital airway stenosis and pneumonia who were admitted to PICU of Beijing Children′s Hospital of Capital Medical University during 5 years and treated with NCPAP within 48 hours after admission.The baseline data, clinical manifestations, vital signs, arterial blood gas, clinical outcomes, NCPAP use time and adverse reactions were collected.Results:A total of 64 children were included in this study, with 58 cases in the effective group and six cases in the ineffective group.The total effective rate of NCPAP was 90.6% (58/64) during 5 years.In the effective group, 63.8% patients were weaned in three to seven days, with an average weaning time of 6.09 days.In the effective group, the heart rate and PaCO 2 after NCPAP treatment were significantly lower than that before treatment, and pH and PaO 2 were significantly higher than that before treatment, and the difference was statistically significant (all P<0.05). A total of six patients in the ineffective group were finally changed to tracheal intubation and invasive ventilation.The survival rate of both groups was 100%.All cases had no adverse reactions or complications. Conclusion:NCPAP can effectively improve the oxygenation in children with congenital airway stenosis and severe pneumonia, with high efficiency and good safety.

Objective:To investigate the effectiveness of ketamine in the treatment of refractory status epilepticus (RSE) and super refractory status epilepticus (SRSE) in children.Methods:A retrospective study was conducted to collect and analyze the medical data of 18 children with RSE or SRSE who received ketamine in intensive care unit of Beijing Children′s Hospital from January 2016 to December 2018. According to the different regimen of ketamine, all children were divided into the loading-maintenance group (7 cases) and the maintenance group (11 cases). According to the control of status epilepticus, the patients were divided into controlled group (11 cases) and non-responsive group (7 cases).Wilcoxon′s rank sum test or Fisher′s exact test were used to compare the effectiveness between groups.Results:There were 9 males and 9 females in the study group, aged 6.7 (4.5, 9.0) years. Seven cases had RSE and the remaining had SRSE. Four cases died during hospitalization. After the initiation of ketamine treatment, RSE and SRSE were controlled in 11 children. The duration of ketamine administration was 4 (2, 11) days. The dose was 2.2 (1.2, 5.3) mg/(kg·h) in all patients, and 2.4 (1.3, 6.0) mg/(kg·h), 2.0 (1.0, 4.0) mg/(kg · h) in the controlled and non-responsive group, respectively ( Z=-0.272, P=0.791). The RSE or SRSE were terminated in all the 7 patients who received loading dose of ketamine, with the dose of 1.5 (0.3,1.6) mg/kg. In the 11 patients who only received maintenance treatment, 4 had the RSE and SRSE terminated, which showed a significantly lower effectiveness than in loading-maintenance group (7/7 vs. 4/11, P=0.01). Regarding the adverse reactions, saliva secretion increased in 8 children during the ketamine administration, otherwise unremarkable. Conclusion:Loading dose followed by maintenance of ketamine can control children′s RSE and SRSE well, without significant adverse reactions.

Objective To analyze the clinical characteristics of fatal cases with confirmed influenza A ( H1N1) in children in order to improve the diagnosis and treatment. Methods The fatal cases of influenza A (H1N1) admitted to Pediatric Intensive Care Unit of Beijing Children′s Hospital from December 2017 to March 2018 were collected,whose clinical features,diagnosis,treatment,and the causes of death were ana-lyzed retrospectively. Results A total of eight children were enrolled. The age ranged from 3 months to 9 years,and six cases were less than 5 years old. Four cases had underlying diseases. All patients had fever, cough,dyspnea,cyanosis,flaring nares and three depressions sign. Four cases had coma. On admission,the average score of pediatric index of mortality was 58. 8％. Blood routine test showed that lymphocyte predomi-nance in six cases, three cases had leukopenia. C-reactive protein elevated in five patients. Procalcitonin increased in all cases. P/F ratio (PaO2/FiO2) were less than 100 mmHg(1 mmHg =0. 133 kPa) in four cases who were diagnosed as severe acute respiratory distress syndrome. The pulmonary imaging showed mul-tiple parenchymal or mixed lesions. Three cases had air leakage syndrome,and one had pleural effusion. Bac-terial culture was performed on the day of admission. Four cases were complicated with bacterial infection. Three cases had Gram-positive cocci infection,and multidrug-resistant bacteria were predominant. The aver-age time from onset to definitive diagnosis was 8 days. Neuraminidase inhibitors were used in all patients,and the average time from onset to the first dose was 8 days. Mechanical ventilation were performed in all patients in this group. Extracorporeal membrane oxygenation was applied in one case. Four cases in this group died of severe acute respiratory distress syndrome. Three cases died of influenza associated encephalopathy,and one died of septic shock with multiple organ failure. Conclusion Fatal influenza A (H1N1) mostly appeares in children under 5 years old or with underlying diseases. Acute respiratory distress syndrome and influenza associ-ated encephalopathy are the main causes of death. Delayed diagnosis and delayed use of anti-influenza drugs may be an important factors leading to death,and bacteria infection may be another important cause of death.

Objective To analyze the cause of fatal cases in children with confirmed influenza virus infection,and in order to improve the level of diagnosis and treatment.Methods Deaths in critical illness of influenza were collected from November 2017 to April 2018 in Pediatric Intensive Care Unit of Beijing Children's Hospital,Capital Medical University.The clinical characteristics and causes of death were retrospectively analyzed according to the different virus types.Results A total of 19 cases were included.Fifteen cases (78.95％) were less than 5 years old and 9 cases (47.37％) were less than 2 years old.On admission,the median score of pediatric index of mortality 2 was 72.7％.There were 11 cases of influenza H1N1 and 8 cases of influenza B.Six cases had underlying diseases.All patients had fever,cough and dyspnea.Thirteen patients had coma.Seventeen cases had pneumonia,11 cases had severe acute respiratory distress syndrome(ARDS),3 cases had air leakage syndrome and 8 cases had influenza-related encephalopathy(IAE).Ten cases (52.63％) died of severe ARDS,7 cases (36.84％) died of IAE,1 case(5.26％) died of multiple organ dysfunction,and 1 case(5.26％)died of severe myocarditis and cardiogenic shock.There was statistical difference in the time from onset to death between the ARDS group and IAE group[15(4,22) d vs.3(2,8) d] (Z =-2.063,P =0.039).Among the children who died of severe ARDS,most patients in influenza H1 N1 group ＜ 2 years old,while those influenza B group ≥ 2 years old.All children who died of IAE were all ≥ 1 years old.Six cases (31.58％) had bacterial infection,mainly gram-positive cocci.All patients were treated with neuraminidase inhibitors.The average time from onset to the first time of medication was 5 days.Conclusions Severe ARDS and IAE are the main causes of death in children with influenza virus infection.Compared with ARDS,the condition of children with IAE worsened more rapidly.

Objective: To evaluate the effect of prophylactic nimodipine in vasospasm prevention and outcome improvement in children with subarachnoid hemorrhage (SAH). Methods: A prospective, randomized controlled clinical trial which enrolled children with SAH who were admitted to pediatric intensive care unit (PICU) of Beijing Children′s Hospital from January 2015 to October 2018 was conducted. A total of 43 patients were randomly divided into nimodipine group (24 patients) and control group (19 patients) according to random number table. Transcranial Doppler (TCD) was used to dynamically monitor blood flow velocity and spectrum monography of bilateral middle cerebral artery (MCA) for vasospasm evaluation. Pediatric cerebral performance category (PCPC) scale was used to evaluate patients′ brain function on 28^th day after discharge. Data were analyzed by t test, Mann-Whitney U test, χ² test. Results: Except heart rate ((157±26) vs. (137±34) beats/min, t=2.079, P=0.045), no significant differences existed between the two groups in basic demographic characteristics, primary diseases, and clinical manifestations (all P>0.05). The peak velocities of bilateral MCA on the 5^th day after admission were significantly lower in nimodipine group (left MCA (136±34) vs. (158±23) cm/s, t=-2.890, P=0.006; right MCA (129±34) vs. (176±27) cm/s, t=-3.717, P=0.001). Likewise, a lower peak velocity of left MCA was observed on the 7^th day after admission in nimodipine group ((127±45) vs. (152±13) cm/s, t=-2.903, P=0.007), but no significant difference existed in that of right MCA ((131±48) vs. (150±22) cm/s, t=-1.760, P=0.090). Eleven patients suffered from vasospasm, 25% (6/24) in nimodipine group and 26% (5/19) in control group (χ²=0.010, P=1.000), within whom 8 patients had complete remission after continuing nimodipine treatment, one died in hospital and the other two′s vasospasm still existed at the time of discharge. No significant differences were found between the two groups in mean length of hospitalization, proportion of mechanical ventilation, Glasgow coma scale at discharge, survival rate at discharge or survival rate on 28^th day after discharge (all P>0.05). However, nimodipine group had a higher proportion of favorable PCPC brain function (92% (22/24) vs. 63% (12/19), χ²=5.208, P=0.030). No side effects such as hypotension, rash or injection site erythema were observed. Conclusion Prophylactic nimodipine cannot reduce vasospasm incidence in children with SAH but may improve short-term brain function, without any significant safety issues.

Objective: By studying the efficacy of interleukin (IL)-6 receptor antagonist (tocilizumab) on acute inflammation of systemin juvenile id-iopathic arthritis (sJIA) and its effect on the downstream signaling pathways and inflammatory factors of IL-6 to further reveal the role of tocilizumab in sJIA. Methods: From December 2015 to December 2018, 64 sJIA children were randomly divided into two groups: 31 cases who were treated with tocilizumab+ glucocorticoid+disease-modifying anti-rheumatic drugs (DMARDs) as the tocilizumab group, 33 cases who were treated with placebo (vitamin C) + glucocorticoid+DMARDs as the control group. They were treated for one year. The levels of IL-2, IL-4, IL-6, IL-10 and tumor necrosis factor (TNF)-α were detected by enzyme-linked immunosorbent assay (ELISA). The expressions of p65 and receptor activator for nuclear factor-κB ligand (RANKL) in peripheral blood mononuclear cells (PBMCs) were detected by quantitative polymerase chain reaction (qPCR). The expressions of signal transducer and activator of transcription (STAT3)/phosphates signal transducer and activator of transcription 3 (p-STAT3)/suppressor of cytokine signaling 3 (SOCS3) before and after treatment were detected by Western blotting. The differences between groups were analyzed by variance analysis. Normal distributed data was tested by K-W test. Twenty normal control subjects came from the pediatric clinic in our hospital. Results: There was no significant difference in the demographic data between the two groups (P>0.05). Among them, 2 children who were treated with tocilizumab dropped out after one month treatment and three months due to un-affordability respectively. The C-reactive protein (CRP), ferritin (FER), erythrocyte sedimentation rate (ESR) in the tocilizumab treatment group decreased significantly after 6 months and 1 year when compared with the disease control group. The concentration of IL-6 in the tocilizumab group (77±46) pg/ml, control group (82±40) pg/ml were higher than that in the healthy control group (10±3) pg/ml (F=4.683, P=0.001; F=2.581, P=0.03). After one year, the concentration of IL-6 (316±42) pg/ml in the tocilizumab group was higher than that in the disease control group (62±40) pg/ml (F=11.2, P=0.001). The expression of RANKL and p65 mRNA in treatment group was significantly higher than that in healthy control group (K-W=10.03, P<0.01; K-W=9.42, P<0.01). After one year, the expression of RANKL and p65 mRNA in treatment group was lower than that in disease control group (K-W=9.964, P<0.01; K-W=10.75, P<0.01). The expression of STAT3/p-STAT3/SOCS3 in disease control group before medication was significantly higher than that in healthy control group, while the expression of p-STAT3/SOCS3 in the treatment group was significantly higher than that in healthy control group. The expression of STAT3/p-STAT3 in the tocilizumab group was significantly lower than that in the disease control group (K-W=12.54, P<0.01; K-W=10.52, P<0.01). Conclusion Tocilizumab can effectively alleviate the symptoms of sJIA in active phase, down-regulate the expression of STAT3/p-STAT3 protein, thereby reducing the transcription of downstream nuclear factor (p65, RANKL) mRNA, thereby affecting the proliferation of synovial cells and reducing bone destruction, but has no significant effect on the secretion of IL-6.

Objective? To design a satisfaction scale on nutrition support nursing service for inpatients, and establish a structural equation model of inpatient satisfaction with nutrition support nursing service in central city area to test the scale, so as to construct a tool for evaluating the satisfaction with nutrition support nursing service. Methods? This study selected 18 indicators affecting the satisfaction with nutrition support nursing services of inpatients based on the European Customer Satisfaction Index (ECSI), the American Customer Satisfaction Index (ACSI) Model, and the China Customer Satisfaction Index Model. From September to December of 2017, a convenience sampling investigation was carried out among the inpatients receiving nutritional support in a Class?Ⅲ Grade A medical institution. Through factor analysis, the 18 impact indicators were categorized into 6 dimensions, namely, perception of nutrition support nursing quality, nursing expectations, nutrient support nursing brands, nutrient support nursing service satisfaction, patient nutrient support nursing loyalty, patient nutrient support nursing complaints. Results? A structural equation model for satisfaction of nursing support services for inpatients was constructed, and the rationality of the 6 dimensions was verified. The Cronbach's α indexes of the 6 dimensions were all equal or greater than 0.80. KMO test coefficient was 0.745. Bartlett spherical test results showed χ2=3 402.132 (P＜0.01). The reasonableness of the six dimensions was verified, and the fitting effect of the model was good (χ2/df=1.416, RMS of residual=0.030, goodness of fit index=0.945, adjusting goodness of fit index=0.927, standard fitting index=0.947, value-added fitting index=0.984, comparing fitting index=0.984, root mean square of approximate error=0.035). Conclusions? With this model, nurse managers can identify the factors affecting the satisfaction of patients receiving nutritional support, and propose continuous improvement strategies that will help improve the effectiveness of nursing services, and improve patient satisfaction with inpatient nutritional support, so as to provide reference for the research of satisfaction model for inpatients.