Objective To explore the advantages and limitations of different evaluation methods for medical quality.Methods The rank sum ratio method(RSR),rank sum ratio method after principal component analysis(RSR-after-PCA),and technique for order preference by similarity to ideal solution(TOPSIS)were used to comprehensively evaluate the surgical medical quality in a hospital,respectively.The results of the three evaluation methods were compared.Results The result of Kendall's coefficient of concordance showed high consistency among the three evaluation methods(Kendall's W=0.879,χ2=26.364,P=0.003),although there were significant differences in the evaluation outcomes for some departments.Conclusion Each evaluation method is capable of objectively evaluating the overall situation of a department,but it also has certain limitations and scope of application.The appropriate method should be selected based on the purpose of analysis and data distribution.The evaluation indexes in hospitals are dispersed,but may be correlated to some extent.RSR-after-PCA can overcome these problems and is more suitable for the comprehensive evaluation of multiple indexes in hospitals.

Objective:To analyze the current status of physical composition in medical workers and the evaluation effects of each indicator on nutritional status.Methods:It was a cross-sectional study. A total of 313 medical workers from one hospital in Beijing were recruited from May 21st to June 22nd in 2021 for body composition measurement. Variance analysis was used to compare the age and gender distribution differences in total body fat, fat-free body weight, body fat percentage, skeletal muscle, protein, water, mineral, and basal metabolic rate indicators among the medical workers. Protein content, body fat percentage, and body mass index (BMI) were used to evaluate the nutritional status of medical workers, and the evaluation effects were analyzed too.Results:Of the 313 medical workers included in this study, there were 221 females and 92 males. In females, total body fat, fat-free body weight, skeletal muscle mass, and basal metabolic rate were all positively correlated with age ( F=19.823, 6.745, 6.561, 6.707) (all P<0.001). There was no significant differences in body composition indicators among male participants of different age groups (all P>0.05). The total body fat in female medical workers was significantly higher than that in males (17.58 kg vs 17.08 kg). Total water, protein, minerals, fat-free body weight, skeletal muscle mass, and basal metabolic rate were all significantly lower in female medical workers when compared with those in males [30.40 kg vs 41.78 kg, 8.07 kg vs 11.26 kg, 2.96 kg vs 3.88 kg, 41.43 kg vs 56.92 kg, 22.37 kg vs 31.98 kg, 1 264.86 kcal (1 kcal=4.184 kJ) vs 1 599.39 kcal] (all P<0.05). Among the medical workers, 190 cases (60.7%) had normal protein levels, 103 cases (32.9%) had protein deficiencies, and 20 cases (6.3%) had protein levels exceeding the standard. Additionally, 110 cases (35.1%) had normal body fat ratios, 193 cases (61.6%) had excessive body fat ratios, and 10 cases (3.2%) had insufficient body fat ratios. Concerning body mass index (BMI), 198 cases (63.2%) had normal BMI. Among them, 145 cases (73.2%) had normal body fat ratios, 53 cases (26.7%) had excessive body fat ratios, 108 cases (54.5%) had normal protein levels, 89 cases (44.9%) had insufficient protein levels, and 1 case (0.5%) had excessive protein levels. Furthermore, 103 cases (32.9%) had excessive BMI, with 20 cases (19.4%) having normal body fat ratios indicating the muscle/bone strength type, and 83 cases (80.6%) having normal protein levels indicating the muscular type. Lastly, 12 cases (3.8%) had insufficient BMI, including 6 cases (50.0%) with normal body fat ratios. Conclusion:There are significant gender differences in body composition distribution among healthcare workers. In females, total body fat, lean body mass, skeletal muscle, and basal metabolic rate are all correlated with age. Evaluating individual nutrition status based solely on BMI would underestimate the obesity rate. Individuals with low or high protein/muscle content should not be assessed using BMI.

Objective:To assess the efficacy of immunosuppressor on treatment of Henoch-Sch?nlein purpura nephritis(HSPN).Methods:Literatures were searched in PubMed, Cochrane library, Web of Science, Wanfang database, CNKI and CBM database from inception to January 2021.The studies that investigated the effect of immunosuppressor on HSPN outcomes were included.Article screening, data extraction and quality assessment were accomplished by two investigators independently, and statistical analyses were performed by STATA 14.Results:Ten studies were included with 443 cases, of which, 245 cases were in the experimental group while 198 cases were in the control group.The Meta-analysis showed that the experimental group had higher complete remission rate( OR=1.95, 95% CI 1.19-3.22, P=0.009), total remission rate ( OR=2.92, 95% CI 1.74-4.88, P<0.001), proteinuria decreasing level ( SMD=0.35, 95% CI 0.09-0.61, P=0.008), the increasing level of serum albumin ( SMD=1.27, 95% CI 0.43-2.11, P=0.003) and the increasing level of estimated glomerular filtration rate ( SMD=0.48, 95% CI 0.21-0.76, P=0.001), lower relapse rate ( OR=0.19, 95% CI 0.05-0.72, P=0.015) as well as death rate ( OR=0.19, 95% CI 0.04-0.78, P=0.021)than those of the control group. Conclusion:The immunosuppressor could enhance complete remission rate, total remission rate, proteinuria decreasing level, the increasing level of serum albumin and the increasing level of estimated glomerular filtration rate, reduce relapse rate and death rate of HSPN patients.

Henoch-Schonlein purpura (HSP) is one of the most common clinical manifestations of systemic small vasculitis in children with non-thrombocytopenic, and mainly manifested as skin purpura, arthritis, gastrointestinal symptoms and Henoch-Schonlein purpura nephritis (HSPN). The severity of renal involvement is the main factor determining the long-term prognosis of children with HSP.Studies have revealed that the determination of pentraxin 3 (PTX3) in serum can be used for early diagnosis of HSPN and prediction of renal injury.In this paper, the origin, gene and protein structure, function, potential relationship and mechanism of action between PTX3 and HSP were discussed, so as to provide new ideas for the early diagnosis and treatment of HSPN.

Objective:To evaluate the prognostic value of amplitude-integrated electroencephalogram(aEEG) on predicting long-term neurodevelopmental outcome of preterm infants.Methods:Literatures were searched in PubMed, the Cochrane library, Web of science, Wanfang database, CNKI and CBM database from inception to August, 2020.The studies which investigated the prognostic value of aEEG on neurodevelopmental outcome of preterm infants were included.Articles screening, data extraction and quality assessment were accomplished by two investigators independently, and statistical analyses were performed by Meta-disc1.4.Results:Six studies were included with 557 cases.The Meta-analysis revealed that the pooled sensitivity was 0.81 (95% CI 0.75-0.87), specificity was 0.68 (95% CI 0.63-0.73), positive likelihood ratio was 2.32(95% CI 1.52-3.52), negative likelihood ratio was 0.27(95% CI 0.19-0.37)and the area under the curve of summary receiver operating characteristic curve was 0.79(95% CI 0.75-0.82). Burst-suppression, continuous low voltage and flat trace were regard as a index of poor neurodevelopmental prognosis. Conclusion:aEEG is a valuable tool for predicting the outcome of long-term neurodevelopment in preterm infants.

Objective:To evaluate the treatment effect of hemoperfusion(HP) on Henoch-Sch?nlein purpura(HSP) outcomes.Methods:PubMed, Cochrane library, Web of science, Wanfang database, CNKI and CBM database were searched from inception to February 2020.Literatures of randomized controlled trials(RCTs) that investigated the effect of HP on HSP outcomes were included.Articles screening, data extraction and quality assessment were accomplished by two investigators independently, and statistical analyses were performed by RevMan 5.3.Results:Thirteen RCTs were included with 803 cases, of which, 397 cases were in the HP group while 406 cases were in the control group.The Meta-analysis revealed the HP group had less disappearing time of hematuresis or albuminuria[ MD=-2.77, 95% CI(-3.18, -2.36), P<0.001], relieving time of abdominal pain[ MD=-1.70, 95% CI(-2.17, -1.23), P<0.001], disappearing time of hematochezia[ MD=-1.54, 95% CI(-1.68, -1.40), P<0.001], and the length of hospital stay[ MD=-3.23, 95% CI(-3.60, -2.87), P<0.001] than the control group. Conclusion:The HP could shorten disappearing time of hematuresis or albuminuria, relieving time of abdominal pain, disappearing time of hematochezia, and the length of hospital stay of HSP.

Objective: To summarize the clinical features of leukoencephalopathy with vanishing white matter disease (VWM) in children. Methods: A retrospective cohort study was performed on 54 genetically diagnosed VWM patients in Peking University First Hospital from January 2007 to March 2019. Paper registration form and electronic medical record system were used to collect the data,and the children were divided into five groups according to the age of disease onset:<1 year, 1-<2 years, 2-<4 years, 4-<8 years and 8-<18 years respectively. The progression of motor function, episodic aggravation, epileptic seizures, survival time, brain magnetic resonance imaging (MRI) and genotype features were analyzed and compared. Non-parametric test, χ² test or Fisher′s exact test were used for comparison among groups; Kaplan-Meier survival curve was adopted to delineate the survival status of the children. Results: Fifty-four VWM patients were included in the study, including 34 males and 20 females.The age of disease onset was 2 years and 8 months (ranged from 6 months to 9 years and 7 months). Onset age was less than 1 year in 5 cases; onset age was 1-<2 years in 12 cases; onset age was 2-<4 years in 25 cases; onset age was 4-<8 years, in 10 cases; onset age was 8-<18 years in 2 cases; 94% (51/54) of patients had complaint of motor regression at the first visit; 87% (47/54) of patients suffered from episodic aggravation. Episodic seizures occurred in 43% (23/54) patients. In survivors with disease durations of 1-3 years, in 38% (9/24) patients the disease was classified as grades Ⅳ-Ⅴ by gross motor function classification system (GMFCS). For the onset age 1-<2 years group, 1 patient was classified as GMFCS Ⅳ among 3 survivors with disease durations of 1-3 years. As for the 2-<4 years group, 6 patients were classified as GMFCS Ⅳ-Ⅴ among 15 patients with disease durations of 1-3 years, whereas 1 patient was classified as GMFCS Ⅳ-Ⅴ among 4 patients with disease durations of 1-3 years in the 4-<8 years group. Lesions, liquefaction and diffusion restriction in brain MRI were compared among different groups, and it was revealed that the earlier the age of disease onset was, the more likely the subcortical white matter (frontal lobe P<0.01,temporal and parieto-occipital lobe both P=0.002), internal capsule (anterior limb P<0.01, posterior limb P=0.00) and brain stem (midbrain P=0.001, pons P<0.01) were to be involved. In addition, internal capsule (anterior limb P=0.002, posterior limb P=0.005) and brain stem (midbrain P=0.001, pons P=0.003) showed more diffuse restricted diffusion. Moreover, the subcortical white matter (frontal and parieto-occipital lobe both P<0.01, temporal lobe P=0.005) showed earlier rarefaction. The 1-year and 2-year survival rates of the overall patients were 81% and 75% respectively, while the 15-year survival rate was 45%. EIF2B5 gene variation was the most common, which accounts for 43% (23/54), followed by EIF2B3 (22%, 12/54). Conclusions The majority of VWM patients complained of motor regression at the first visit, episodic aggravation and epileptic seizures are common in the course. Earlier age at onset is associated with more rapid clinical progression, shorter survival time as well as more extensive lesions, liquefaction and diffusion restriction in brain MRI. The most common variant gene is EIF2B5, followed by EIF2B3.

To summarize the clinical features of leukoencephalopathy with vanishing white matter disease (VWM) in children. Methods A retrospective cohort study was performed on 54 genetically diagnosed VWM patients in Peking University First Hospital from January 2007 to March 2019. Paper registration form and electronic medical record system were used to collect the data,and the children were divided into five groups according to the age of disease onset:<1 year, 1-<2 years, 2-<4 years, 4-<8 years and 8-<18 years respectively. The progression of motor function, episodic aggravation, epileptic seizures, survival time, brain magnetic resonance imaging (MRI) and genotype features were analyzed and compared. Non?parametric test, χ2 test or Fisher's exact test were used for comparison among groups;Kaplan?Meier survival curve was adopted to delineate the survival status of the children. Results Fifty?four VWM patients were included in the study, including 34 males and 20 females.The age of disease onset was 2 years and 8 months (ranged from 6 months to 9 years and 7 months). Onset age was less than 1 year in 5 cases; onset age was 1-<2 years in 12 cases; onset age was 2-<4 years in 25 cases; onset age was 4-<8 years, in 10 cases; onset age was 8-<18 years in 2 cases; 94% (51/54) of patients had complaint of motor regression at the first visit; 87% (47/54) of patients suffered from episodic aggravation. Episodic seizures occurred in 43% (23/54) patients. In survivors with disease durations of 1-3 years, in 38% (9/24) patients the disease was classified as grades Ⅳ?Ⅴ by gross motor function classification system (GMFCS). For the onset age 1-<2 years group, 1 patient was classified as GMFCSⅣamong 3 survivors with disease durations of 1-3 years. As for the 2-<4 years group, 6 patients were classified as GMFCS Ⅳ-Ⅴamong 15 patients with disease durations of 1-3 years, whereas 1 patient was classified as GMFCS Ⅳ-Ⅴ among 4 patients with disease durations of 1-3 years in the 4-<8 years group. Lesions, liquefaction and diffusion restriction in brain MRI were compared among different groups, and it was revealed that the earlier the age of disease onset was, the more likely the subcortical white matter (frontal lobe P<0.01,temporal and parieto?occipital lobe both P=0.002), internal capsule (anterior limb P<0.01, posterior limb P=0.00) and brain stem (midbrain P=0.001, pons P<0.01) were to be involved. In addition, internal capsule (anterior limb P=0.002, posterior limb P=0.005) and brain stem (midbrain P=0.001, pons P=0.003) showed more diffuse restricted diffusion. Moreover, the subcortical white matter (frontal and parieto?occipital lobe both P<0.01, temporal lobe P=0.005) showed earlier rarefaction. The 1?year and 2?year survival rates of the overall patients were 81% and 75% respectively, while the 15?year survival rate was 45%. EIF2B5 gene variation was the most common, which accounts for 43% (23/54), followed by EIF2B3 (22%, 12/54). Conclusions The majority of VWM patients complained of motor regression at the first visit, episodic aggravation and epileptic seizures are common in the course. Earlier age at onset is associated with more rapid clinical progression, shorter survival time as well as more extensive lesions, liquefaction and diffusion restriction in brain MRI. The most common variant gene is EIF2B5, followed by EIF2B3.

Objective To analyze the clinical and imaging features of hypomyelination with brain stem and spinal cord involvement and leg spasticity (HBSL) due to mutations in DARS,and to identify DARS mutations responsible for HBSL.Methods Data on 2 HBSL patients who were admitted to the pediatric department of Peking University First Hospital from January 2009 through December 2016 were reviewed and the 2 patients were followed up.Targeted next generation sequencing,whole exome sequencing and Sanger sequencing were employed to identify potential genetic variations of the children and their parents.The clinical manifestations,MRI features and genotypic characteristics of two patients were reviewed,and the literature was reviewed.HBSL reported cases were searched with"leukoencephalopathies,DARS" on databases of PubMed,Wanfang,China National Knowledge Infrastructure and VIP from 1975 to 2017.The clinical manifestations and molecular features were analyzed.Results Both patients showed delayed motor development,but had normal cognitive development.At the age of 8 years,case 1 reached the most significant motor development milestone of only standing with help during the last follow-up.At the age of 9,case 2 could walk independently during the last follow-up.On physical examination,both showed leg spastcity,active tendon reflex,positive Babinski sign.Both patients had brain MRI findings of high T2WI signal in bilateral deep cerebral white matter,slightly lower T1WI,and no abnormal DWI signal.Lesions of case 1 were relatively extensive and involved subcortical white matter,corpus callosum and internal capsule.Spinal MRI scans for both patients showed no abnormal signals.Novel mutations in DARS gene-namely,c.1498_1499insTCA (p.500_501insIle) and c.1210A＞G (p.Met404Val),c.1432A＞G (p.Met478Val) and c.1210A＞G (p.Met404Val)were identified in case 1 and case 2 respectively.On the database,2 reports involving 13 foreign patients were retrieved.The age of disease onset was from 4 months to 18 years,and their initial symptoms were development delay or regression.Most of them presented with progressive lower extremity spasm,and the brain magnetic resonance imaging was characterized by hypomyelination in white matter.Clinical phenotypes of different age groups were significantly different.Conclusion We have reported two patients with HBSL in China,and 3 novel mutations in DARS,which is helpful for the diagnosis and genetic counseling of HBSL.

Objective: To explore the phenotypic and genotypic characteristics in Chinese children with classic pantothenate kinase-associated neurodegeneration (PKAN). Method: The clinical, radiographic and genetic data of all PKAN patients diagnosed at pediatric department of Peking University First Hospital from November 2006 to December 2016 were retrospectively collected and analyzed. Result: Twenty patients with classic PKAN were included in the study. The median age at onset was 3.5 years (ranging from 1.0 to 10.0 years), and the most common initial symptom was gait disturbance (16 cases). At the last evaluation, the clinical features were limbs dystonia (20 cases), dysarthria (16 cases), dysphagia (11 cases), pyramidal sign (7 cases), mental regression (3 cases) and pigmentary retinopathy (5 cases). For those classic PKAN patients, the median time from onset of disease to loss of independent ambulation was 6.9 years (ranging from 2.0 to 12.0 years). Imaging data showed, except "eye of tiger" in MRI (19 cases), globus pallidus calcification in CT was also found in four patients. In gene testing, 26 different mutations in PANK2 gene were identified, and 16 of 26 were novel mutations. Moreover, c. 1502T>C (p.Ile501Asn) was the most common mutation (4 cases). Conclusion Dystonia is the major neurologic feature of classic PKAN. Disease progression is rapid, with loss of independent ambulation within 10 years after onset. Except "eye of tiger" in MRI, globus pallidus calcification in CT may be another imaging feature of PKAN.Sixteen novel mutations of PANK2 gene were identified in the study.