Objective:To explore therapeutic effect of noninvasive bilevel positive airway pressure ventilation (Bi- PAP)on aged patients with acute left heart failure (ALHF)and evaluation value of NT-proBNP for therapeutic effect in these patients.Methods:A total of 80 aged ALHF patients treated in our hospital were selected.According to random number table,they were equally divided into routine treatment group and BiPAP group (received BiPAP therapy based on routine treatment group ).NT-proBNP level,heart rate (HR),systolic blood pressure (SBP),di- astolic blood pressure (DBP),respiratory rate (RR)and arterial blood gas were compared between two groups be- fore and after treatment.Linear correlation analysis was used to evaluate the correlation among plasma NT-proBNP level and above indexes.Results:There were no significant difference in all indexes between two groups before treatment (P>0.05 all).Compared with before treatment,on 4h after treatment,there were significant reductions in levels of HR,SBP,DBP,RR and NT-proBNP,and significant rise in partial pressure of oxygen in artery (PaO2 ) in both groups (P<0.05 or<0.01),and compared with routine treatment group,there was significant rise in PaO2 [(75.13±19.93)mmHg vs.(85.88±18.47)mmHg],and significant reduction in NT-proBNP level [(3786.71± 1270.38)pg/ml vs.(2658.65±1222.08)pg/ml]in BiPAP group (P<0.05 both).Linear correlation analysis indi- cated that NT-proBNP level was significant positively correlated with HR (r=0.383),SBP (r=0.360),RR (r=0.345)and PaCO2 (r=0.413),and significant inversely correlated with PaO2 (r=-0.471),P<0.05 or<0.01. Conclusion:BiPAP ventilation therapy can improve clinical therapeutic effect in aged ALHF patients,and NT-proB- NP is help to evaluating its therapeutic effects.

Objective To investigate the statistical.method for analyzing nurse job burnout Data. Methods Hotelling T2 test and multivariate analysis of variance(MANOVA)were used to analyze nurse job burnout datum. Results There was statistical significance between different cities,different ethnieities in the personal achievements,hut from three dimensions of the whole job burnout,there are no statistical sig-nificant differences among different cities, different ethnicities and different ages. Conclusions The use of Hotelling T2 test and multivariate analysis of variance to analyzing nurse job burnout data, not only ob-tain overall conclusion, and further use of single-variable analysis may also gain comparative results of each dimensions.

Objective To investigate the relationship between correlative factors of ambulatory arterial stiffness in-dex (AASI) and target organ damage (TOD) in patients with primary hypertensive. Methods A total of 330 hypertensive pa-tients were included in the study and divided into two groups according to the value of AASI:low AASI group (n=167) and high AASI group (n=163). The value of AASI was obtained from 24-hour ambulatory blood pressure monitor (ABPM). The clinical data were collected including general information, the data of ABPM, results of coronary angiography, left ventricular mass index (LVMI), estimated glomerular filtration rate (eGFR) and ankle brachial index (ABI) in two groups. Results There were significantly higher values of age (years:64.91 ± 9.70 vs 59.12 ± 10.00), the proportion of diabetes (33.8% vs 14.8%), the proportion of non-dipper patterns of hypertension (65.6%vs 43.7%), 24-hour pulse pressure (PP, mmHg:65.27± 11.31 vs 56.06±10.51), 24-hour diastolic blood pressure standard deviation(DBPSD, mmHg:9.64±2.47 vs 8.31±2.31), the number of coronary artery lesions (1.78±1.10 vs 1.27±1.07), LVMI (g/m2:125.74±29.65 vs 107.69±23.23) and the proportion of peripheral vascular disease (27.3%vs 16.4%) in high AASI group than those in low AASI group (P<0.01). The level of eGFR was significantly lower in high AASI group than that in low AASI group [mL/(min · 1.73 m2):85.31 ± 20.31 vs 99.67 ± 17.76]. There were positive correlation between AASI and coronary lesions (r=0.235), LVMI(r=0.168) and peripheral vascu-lar disease (r=0.167). And there was a negative correlation between AASI and eGFR (r=-0.187). The multiple linear regres-sion analysis showed that age, diabetes, PP, DBPSD and non-dipper patterns of hypertension were the predictors of AASI. Conclusion AASI correlated with age, diabetes, PP, blood pressure variability and non-dipper patterns of hypertension. The higher level of AASI may relate to the development of TOD in patients with primary hypertensive.

Objective To investigate the influence and clinical significance of single and dual-chamber pacing on central aortic pressure (CAP) and augmentation index (AI) in non-smoking individuals. Methods Totally, 83 non-smokers with pacemaker-implanted were consecutively enrolled in this study, and they were divided into three groups:dual-chamber pacemaker group (DDD, n=35), single-chamber pacemaker group (VVI, n=33) and control group (n=15). Heart rate (HR), CAP, AI, systolic blood pressure (SBP) and diastolic blood pressure (DBP) were measured in three groups of patients. Finally, DDD pacing mode was turned into VVI pacing mode in patients of DDD group and the indexes were measured again. All of the indexes were recorded and analyzed. Results There were no significant changes in baseline characteristics and laboratory data between three groups (P>0.05). Left atrial diameters were significantly higher in VVI group than those of control group (P<0.05). Values of CAP were significantly higher in DDD group than those of control group and VVI group (P<0.05). Values of AI, corrected AI (AIc) and brachial BP were significantly higher in DDD group than those of VVI group (P<0.05). Values of CAP and brachial BP were significantly lower in VVI group than those of control group (P<0.05), while no significant changes were found in AI and AIC between VVI group and control group (P>0.05). All of these indexes (CAP, AI and brachial BP) were significantly reduced after the pacing mode was changed (P<0.05). Conclusion In non-smokers, dual-chamber pacing mode can increase CAP and AI.

Objective To explore MR perfusion imaging characteristics of rabbit liver.Methods MR perfusion imaging was performed in 10 New Zealand rabbits)weight:2.5～3.0 kg)respectively.The MR perfusion imaging protocol consisted of T1-weighted fast field echo(FFE)sequences with a field of view of 355 mm×75 mm,matrix 89×256,TR/TE of 4.3/1.4 millisecond,slice thickness of 5 mm,intersection gap of 1 mm,NSA of 1.This sequence was repeated 65 times,in 4 slices(in total,80 seconds).The original data were transmitted to PHILIPS workstation and formed MR perfusion images automatically.The MR perfusion images in different liver tissues were observed and analyzed.Results The rabbit liver profiles were depicted on hepatic blood flow(HBF)and hepatic blood volume(HBV)images.Time-intensity curves derived from ROIs drawn in abdominal aorta appeared as a type of rapid increase and decrease,gradually increase in hepatic vein and slowly increase and decrease in normal liver tissues.The peak of MR signal intensity of abdominal aorta,hepatic vein and normal liver tissues were 496±131,323±92,194±58 separately.Conclusion MR perfusion imaging can be performed for rabbit liver successfully by using MR TFE series.The rabbit liver profiles are depicted by HBF and HBV images.The hemodynamics of different liver tissues can be responded by time-intensity curves preliminarily.

Objective: To study the correlation between MR perfusion imaging and pathology after transcatheter arterial chemoembolization (TACE) using VX2 liver cancer model and to provide a theoretical basis to evaluate the curative effect of TACE. Methods; Fifteen New Zealand white rabbits (weight: 2.5-3.0kg) were randomly divided into three groups, with 5 in each group (group 1, pre-TACE; group2, 3 days after TACE; group 3,1 week after TACE). The rabbit VX2 hepatic carcinoma models were presented in all rabbits. All of the three groups received TACE at three weeks after the tumor was implanted. The MR perfusion imaging was performed before chemoembolization, at 3 days and 1 week after chemoembolization respectively for group 1, 2 and 3. Each animal was then sacrificed for pathology observation after MR examination. Results: The lesions assessed before TACE were hyperintense compared with the surrounding liver parenchyma on DWI images. The volume of neoplastic cells became large. Nucleus was hypertrophic with different size and shape. Phase of nucleous mitosis showed in many cells and necrosis was hardly seen. No obvious difference was found between the peripheral area and the core area. At 3 days after TACE, the heterogeneous hypo-intense was observed on DWI images. Many nuclear fragmentation and caryolysis appeared on pathology. Neoplasm necrosis was seen. At 1 week after TACE, the heterogeneous hypo-intense areas became larger. Light microscopy showed incomplete necrosis. There were increased karyopycnosis and nuclear fragmentation. Conclusion: MR perfusion imaging of VX2 liver cancer corresponds well with pathology and can reflect the outcome of liver cancer after TACE.

AIM:To investigate the alleviating effect of ethyl acetate extract from Platycladus orientalis leaves(EAEPOL)on diabetic cardiomyopathy(DCM)and its mechanisms.METHODS:Healthy adult C57BL/6 mice were ran-domly divided into normal control group,DCM group,and EAEPOL group.Cardiac structure and function of the mice were assessed by echocardiography.Myocardial fibrosis was evaluated though myocardial hydroxyproline content determi-nation,myocardial Masson and Sirius red staining,and collagen type I(Col I)and collagen type Ⅲ(Col Ⅲ)immunohis-tochemistry.The degree of myocardial oxidative stress was assessed by measuring malondialdehyde(MDA),superoxide dismutase(SOD)and total antioxidative capacity(T-AOC)levels using kits,as well as detection of nuclear factor E2-re-lated factor-2(Nrf-2)and heme oxygenase-1(HO-1)expression by qRT-PCR and Western blot.Endothelial-mesenchy-mal transition(EndMT)was evaluated by detecting CD31 and α-smooth muscle actin(α-SMA)protein expression by Western blot,and cadherin 5(CDH5)and fibroblast specific protein 1(FSP1)mRNA expression by qRT-PCR,as well as α-SMA immunofluorescence staining.RESULTS:(1)Mouse echocardiography revealed that compared with normal control group,heart rate(HR)and ejection fraction(EF)were significantly reduced in DCM group(P<0.05 or P<0.01),while left ventricular anterior wall thickness at systole and diastole(LVAWs and LVAWd)and left ventricular pos-terior wall thickness at systole and diastole(LVPWs and LVPWd)were significantly increased(P<0.05).Compared with DCM group,the mice in EAEPOL group showed significant increases in HR and EF(P<0.01),and marked decreases in LVAWs,LVAWd,LVPWs and LVPWd(P<0.05).(2)Compared with normal control group,the content of hydroxypro-line in mouse myocardium,the collagen area ratio shown by Sirius red and Masson staining,and the immunohistochemical positive area ratio of Col I and Col Ⅲ in DCM group were significantly increased(P<0.01).Compared with DCM group,the above myocardial fibrosis indicators in EAEPOL group were significantly reduced(P<0.05 or P<0.01).(3)Com-pared with normal control group,the myocardial MDA content and the expression of Nrf-2 in DCM group were significantly increased,while the SOD activity,the T-AOC and the expression of HO-1 was significantly decreased(P<0.01).Com-pared with DCM group,the myocardial MDA content in EAEPOL group was significantly reduced,while the SOD activity,the T-AOC,and the HO-1 and Nrf-2 expression were significantly enhanced(P<0.05 or P<0.01).(4)Compared with normal control group,the myocardial expression of CD31 and CDH5 in DCM group was significantly reduced,the expres-sion of α-SMA and FSP1 was significantly enhanced(P<0.05 or P<0.01),and the α-SMA positive area ratio by immuno-fluorescence staining was also increased(P<0.01).Compared with DCM group,EAEPOL significantly up-regulated the expression of CD31 and CDH5 and down-regulated the expression of α-SMA and FSP1,and the α-SMA positive area ratio by immunofluorescence staining was evidently decreased(P<0.05 or P<0.01).CONCLUSION:EAEPOL may attenuate myocardial fibrosis and improve cardiac function in DCM mice by suppressing oxidative stress and alleviating EndMT.

Objective:To explore the diagnosis and treatment of adolescence-onset methylenetetrahydrofolate reductase (MTHFR) deficiency.Methods:This was a retrospective case study. Nine patients with adolescence-onset MTHFR deficiency were diagnosed at Peking University First Hospital from January 2016 to December 2022, and followed up for more than 1 year. Their general information, clinical manifestations, laboratory tests, cranial images, MTHFR gene variants, diagnosis, treatment, and outcome were analyzed retrospectively.Results:The 9 patients came from 8 families. They had symptoms at age of 8.0 years to 17.0 years and diagnosed at 9.0 years to 17.5 years. Eight were male and 1 was female. Two patients were brothers, the elder brother developed abnormal gait at 17.0 years; and the younger brother was then diagnosed at 15.0 years of age and treated at the asymptomatic stage, who was 18.0 years old with normal condition during this study. The main manifestations of the 8 symptomatic patients included progressive dyskinesia and spastic paralysis of the lower limbs, with or without intellectual decline, cognitive impairment and behavioral abnormalities. Totally, 15 variants of MTHFR gene were identified in the 9 patients, including 8 novel variants. Five patients had brain image abnormalities. Increased plasma total homocysteine level (65-221 μmol/L) was found in all patients, and decreased to 20-70 μmol/L after treatment with betaine and calcium folinate. Besides, the 8 symptomatic patients had their behavior and cognitive problems significantly improved, with a legacy of lower limb motor disorders.Conclusions:Late-onset MTHFR deficiency can occur in adolescence. The diagnosis is usually delayed because of non-specific clinical symptoms. The test of blood total homocysteine could be used as a selective screening test. Eight novel varients of MTHFR gene were identified. Timely treatment can improve clinical condition significantly, and pre-symptomatic treatment may prevent brain damage.

Objective:To analyze the clinical features, genetic characteristics, treatment and follow-up results of patients with hydrocephalus caused by methylmalonic acidemia combined with homocysteinuria, and to discuss the optimal strategies for assessing and treating such patients.Methods:From January 1998 to December 2020, 76 patients with hydrocephalus due to methylmalonic acidemia combined with homocysteinuria in the Department of Pediatrics in 11 hospitals including Peking University First Hospital were diagnosed by biochemical, genetic analysis and brain imaging examination. The patients were divided into operation-group and non-operation-group according to whether they underwent ventriculoperitoneal shunt. The clinical features, laboratory examinations, genotype, and follow-up data were retrospectively analyzed. Data were compared between the two groups using rank sum test, and categorical data were compared using χ 2 test. Results:Among the 76 patients (51 male, 25 female), 5 were detected by newborn screening, while 71 were diagnosed after clinical onset, 68 cases (96%) had early-onset, 3 cases (4%) had late-onset. The most common clinical manifestations of 74 cases with complete data were psychomotor retardation in 74 cases (100%), visual impairment in 74 cases (100%), epilepsy in 44 cases (59%), anemia in 31 cases (42%), hypotonia or hypertonia in 21 cases (28%), feeding difficulties in 19 cases (26%) and disturbance of consciousness in 17 cases (23%). Genetic analysis was performed in 76 cases, all of whom had MMACHC gene variations, including 30 homozygous variations of MMACHC c.609G>A. The most common variations were c.609G>A (94, 62.7%), followed by c.658_660del (18, 12.0%), c.567dupT (9, 6.0%) and c.217C>T (8, 5.3%). Therapy including cobalamin intramuscular injection, L-carnitine and betaine were initiated immediately after diagnosis. A ventriculoperitoneal shunt operation was performed in 41 cases (operation group), and 31 patients improved after metabolic intervention (non-operation group). There was no significant difference in the age of onset, the age of diagnosis, the blood total homocysteine, methionine, and urinary methylmalonic acid concentration between the two groups (all P>0.05). The symptoms of psychomotor development, epilepsy, and visual impairments improved gradually after a long-term follow-up in the operation group. Conclusions:Hydrocephalus is a severe complication of methylmalonic acidemia combined with homocysteinuria. The most common clinical manifestations are psychomotor retardation, visual impairment, and epilepsy. It usually occurs in early-onset patients. Early diagnosis and etiological treatment are very important. Hydrocephalus may improve after metabolic intervention in some patients. For patients with severe ventricular dilatation, prompt surgical intervention can improve the prognosis.

Objective:To analyze the clinical features and CBS gene variants of 13 patients with classic homocystinuria, and the strategies of individual treatment and prevention were explored.Methods:The general information, clinical manifestations, laboratory tests, cranial images, CBS gene variants, diagnosis and therapeutic strategies of 13 patients with classic homocystinuria admitted to the Department of Pediatrics of Children′s Hospital Affiliated to Zhengzhou University and Peking University First Hospital from November 2013 to June 2021 were analyzed retrospectively.Results:There were 13 patients diagnosed at the age of 10 days to 14 years, 6 were male and 7 were female. There were 3 patients detected by newborn screening and received treatment at the asymptomatic stage. There were 10 patients clinically diagnosed at the age of 5 to 14 years. Their symptoms appeared at age of 1 to 6 years. The major clinical manifestations were marfanoid features, lens dislocation and (or) myopia, developmental delay, osteoporosis, and cardiovascular diseases. Brain magnetic resonance imaging showed asymmetric infarcts in 4 patients and hypomyelination in 1 case. Increased blood methionine, plasma total homocysteine and urinary total homocysteine with normal urinary methylmalonic acid were found in 13 patients. The biochemical features were consistent with classic homocystinuria. Totally 18 variants were identified in CBS gene of 13 patients, 10 variants were novel and 8 were reported. only 1 patient was partially responsive to vitamin B 6 treatment, while 12 cases were non-responsive. They were mainly treated with low methionine diet and betaine supplement. Three vitamin B 6 non-responsive cases received liver transplantation at age of 3, 8 and 8 years, respectively. Their blood methionine and total homocysteine returned to normal within a week after liver transplantation. One patient died. Prenatal diagnosis was performed for a fetus when the mother was pregnant again. Two pathogenic CBS gene variants were identified from the amniocytes as same as the proband. Conclusions:The clinical manifestations of classic homocystinuria are complex and variable. Blood amino acid analysis, serum or urine total homocysteine assay and gene analysis are critical for its diagnosis. There were 10 novel CBS gene varients were identified expanding the CBS gene varient spectrum. Liver transplantation is an effective treatment. Prenatal diagnosis is important to prevent classic homocysteinuria.