Objective To evaluate the right heart dysfunction in SLE patients with/ without pulmonary arterial hypertension (PAH) using the parameters recommended by the American Society of Echocardiography (ASE),and to examine whether the right heart dysfunction is directly related to elevated pulmonary arterial systolic pressure(PASP) and pulmonary vascular resistance(PVR).Methods Study population composited of 43 patients with SLE.The patients were divided into two groups according to the PASP measured by echocardiography:Group A was 24 patients with PASP ≤35 mm Hg,Group B was 19patients with PASP＞35 mm Hg.Twenty-two healthy subjects with age and gender matched were set as control group.Routine transthoracic echocardiography study was performed on all patients and 26 parameters were measured in order to compare the differences of the parameters among the three groups.Results There were significant differences in 17 parameters among the three groups (P ＜0.05).Tricuspid annular plane systolic excurtion(TAPSE) and E/A ratio of tricuspid flow velocities in SLE patients without PAH significantly decreased compared to control group (P =0.04 and 0.03).There were significant differences in 11 parameters between SLE with and without PAH group (P ＜0.05).Multivariate logistic regression analysis indicated that after adjustment for age and gender,the patients with elevated PVR associated with a 6.18-fold increase in right ventricular dysfunction compared to the patients with normal PVR (P =0.02).Conclusions The impairment of right ventricular function in SLE patients was directly related to PVR and PASP.Elevation of PVR was an important predictor for right heart dysfunction.

Objective To establish blood purification system for rats and provide a safe and reliable experimental platform for further research of blood purification. Methods The right carotid artery and the contralateral jugular vein of adult male Sprague?Dawley rats were cannulated to creat vascular access for blood purification, by which continuous arteriovenous hemofiltration blood purification system was established. Blood flow, substitution fluid flow and ultrafiltration rate were regulated by rotary mini?pumps. Blood purification therapy continued for 4 hours on the basis of maintained anesthesia and effective anticoagulation. The safety of continuous arteriovenous hemofiltration blood purification systems was evaluated by comparing the arterial blood gas, electrolyte indexes and blood glucose during the blood purification therapy. Closely monitoring the vital signs of rats, such as blood pressure and heart rate, and observing whether there were any side effects, such as massive haemorrhage, thrombogenesis and gas embolism in the therapeutic process. Results There were no obvious changes of arterial blood gas, electrolyte indexes and blood glucose during the blood purification therapy (P>0.05). The vital signs did not fluctuate acutely before and after the blood purification therapy (P>0.05). The incidence rate of side effects was very low. Conclusions
Continuous arteriovenous hemofiltration blood purification system had no obvious adverse effects on healthy rats. Our blood purification system for rats appears to be safe and reliable.

Objective To evaluate the effects of fructose-1 ,6-diphosphate (FDP) pretreatment on lung injury induced by hepatic cold liver ischemia-reperfusion in rats .Methods Eighteen healthy male Sprague-Dawley rats were randomly divided into 3 groups (n= 6 each) using a random number table :sham operation group (S group) ,hepatic cold liver ischemia-reperfusion model group (M group) ,and FDP pretreatment group (FP group) . The animals were anesthetized with intraperitoneal chloral hydrate and kept spontaneous breathing .Laparotomy was performed ,and the related blood vessels were only isolated in group S .Hepatic cold ischemia-reperfusion was induced in M and FP groups .In FP group ,FDP 250 mg/kg was injected via the caudal vein at 15 min before skin incision .At 6 h of reperfusion ,the bronchoalveolar lavage fluid (BALF) was collected to detect the levels of tumor necrosis factor-α(TNF-α) ,interleukin-10 (IL-10) and nitric oxide (NO) by ELISA .Lungs were removed for microscopic examination of the pathological changes by light microscopy .Real-time PCR was used to detect the expression of iNOS mRNA .Results Compared with S group , the levels of TNF-α and NO in BALF were significantly increased , the expression of iNOS mRNA was up-regulated , and the level of IL-10 in BALF was decreased in M and FP groups ( P<0.05 ) .Compared with M group ,the levels of TNF-αand NO in BALF were significantly decreased ,the expression of iNOS mRNA was down-regulated ,and the level of IL-10 in BALF was increased in FP group ( P<0.05 ) .The pathological changes of lungs were significantly attenuated in FP group as compared with M group .Conclusion FDP pretreatment can obviously attenuate lung injury induced by hepatic cold ischemia-reperfusion in rats ,and inhibition of iNOS expression ,reduction of NO synthesis ,and decrease in inflammatory responses are involved in the mechanism .

Objective:To analyze the characteristics of the ocular vestibular evoked myogenic potential on three different eye positions and to explore the appropriate eye position for oVEMP.Methods:15 patients (30 ears) with vertigo who underwent oVEMP test from December 2017 to May 2018 were selected as the patient group, including seven males and eight females, with an average age of (51±13) years. Another 22 (44 ears) healthy young people were recruited into the control group, including 10 males and 12 females, with an average age of (23±5) years. oVEMPs were measured on the following three eye positions respectively: 30 degrees straight up(upper median position),45 degrees upper right(upper right position), and 45 degrees upper left(upper left position). oVEMP elicitation rate, oVEMP latencies, amplitudes and interaural amplitude asymmetry ratio were analyzed by SPSS 23.0 statistical software.Results:There was no statistical significance ( P>0.05) in the oVEMP elicitation rate, oVEMP latency, amplitude and asymmetry ratio on the three eye positions among the control group, the patient group and the overall subjects. Conclusions:The three eye positions can be used to detect oVEMP in clinic. There is no difference in the extraction rate and waveform characteristics. When one of the eye positions is difficult to gaze or not easy to obtain the coincidence curve, the other two can be used to obtain the ideal oVEMP curves as well.

IKBKG is the essential modulator for nuclear factor-κB(NF-κB) signaling pathway, and mutations within this gene can lead to anhidrotic ectodermal dysplasia and immunodeficiency (EDA-ID). Here we report a male patient, who presented with mild frontal bossing, sparse hair, skin pigmentation, conical teeth, and recurrent infections involving bacteria, fungi, and viruses after one month of age, together with hypogammaglobulinemia. These symptoms were consistent with the phenotype of EDA-ID. Genetic analysis revealed a hemizygous mutation c.1249T > G (p.Cys417Gly) in exon 10 of the IKBKG gene in the patient. The mother of the patient was identified as heterozygous carriers of the same mutation. Immunological assessment revealed a significant reduction in memory B cells. The patient showed no skewed TCR diversity. PHA-induced T-cell proliferation was impaired. IFN-γ secretion by Th cells was significantly reduced after PHA stimulation. IκBα degradation rate retained the same in the patient. We summarized the clinical features of the patient and conducted immunological analysis, in order to increase pediatricians′awareness of this rare disease. For boys with early-onset recurrent infections together with ectodermal dysplasia, IKBKG mutation should be considered. In addition, genetic analysis is needed to exclude pseudogene interference and functional evaluations are required.