Objective To report a Chinese boy suffering from nephrotic syndrome associated with Schimke immuno-osseous dysplasia (SIOD). Methods The clnical data and pathological changes of renal biopsy were analyzed and associated literatures were reviewed. The clinicopathological features and diagnosis of SIOD were discussed. Results The first symptom of the patient was recurrent infections. Growth retardation, spondyloepiphyseal dysplasia accompanied by nephrotic syndrome and defective cellular immunity were seen as clinical features in this patient. Renal pathology showed focal segmental glomerulosclerosis. Conclusion Combining the clinical manifestation with renal pathology, the case is diagnosed as Schimke immuno-osseous dysplasia.

Objective To explore the effects of peroxisome proliferator-activated receptorγ (PPARγ) agonist rosiglitazone and 15-deoxy-delta-12,14-prostaglandin J2 (15d-PGJ2) on the expression of PPARγ, toll-like receptor 4 (TLR4) and the activation of STAT1 as well as the local inflammation reaction of abdominal cavity in sprague dawley (SD) rats with peritoneal dialysis- related acute peritonitis induced by lipopolysaccharide (LPS). Methods Twenty-four male SD rats were equally randomized to four groups(n=6 each): control group, injected with 4.25% dextrose peritoneal dialysate (PDF) via abdominal cavity(90 ml/kg); LPS group, injected with LPS(1 mg/kg) via abdominal cavity 4 hours later follewed by PDF injection; rosiglitazone plus LPS group (Rosi group), preconditioned with rosiglitazone (20 mg·kg-1·d-1) by intragastric way for 3 days, then injected with LPS and PDF via abdominal cavity; 15d-PGJ2 plus LPS group (15d-PGJ2 group), preconditioned with 15d-PGJ2 (0.3 mg·kg-1·d-1)via abdominal cavity injection for 3 days, then injected with LPS and PDF via abdominal cavity. The rats were killed 4 hours after PDF injection, IL-6 level in abdominal dropsy was determined by ELISA. Peritoneum tissue was stained by Masson. Leucocyte count in abdominal dropsy was performed. The mRNA expression of PPARγ and TLR4 in peritoneum tissue was determined by RT-PCR; the protein expression of PPARγ, TLR4, p-STAT1 and STAT1 in peritoneum tissue was analyzed by Western blot. Results IL-6 level of abdominal dropsy in LPS group [median 268.53 (range 201.87-335.19) ng/L] was significantly higher than that of control group [median 147.62 (range 130.60-164.64) ng/L] (P<0.01). The IL-6 level of abdominal dropsy in Rosi group [median 110.20 (range 77.60-142.80) ng/L] was significantly lower than that of LPS group (P<0.05). Compared to that of control group, the edematous degree of peritoneum in LPS group was significantly severer, meanwhile, mRNA and proteins expression of PPARγ and TLR4 in rat peritoneum were also significantly higher (P<0.05, P<0.01). Compared to that of LPS group, the edematous degree of peritoneum in Rosi group was lighter, the expression of PPARγ and TLR4 mRNA was significantly up-regulated (P<0.05), meanwhile their proteins expression was down-regulated (P<0.05); and in 15d-PGJ2 group, the edematous degree of peritoneum, the expression of PPARγ mRNA and protein was also decreased (P<0.05), but TLR4 mRNA expression was up-regulated (P<0.01), however, its protein expression was down-regulated (P<0.05). There were no significant differences in leucocyte count of abdominal dropsy among the four groups. The p-STAT1 expression in the rats peritoneum induced by LPS was markedly increased by both rosiglitazone and 15d-PGJ2 (P<0.01). Conclusions Both rosiglitazone and 15d-PGJ2 can down-regnlate the inflammatory reaction in rat peritonitis induced by LIPS, which may be involved in modulating the expression of associated functional protein during LPS signal pathway.

ated acute peritonitis induced by LPS in rats.

Objective To investigate the effects of CD4+CD25high regulatory T cells(Treg)and the imbalance of helper T lymphocyte subsets(Th1/Th2)on the immunological mechanism of IgA nephropathy(IgAN)patients. Methods The percentage of Treg and helper T cells subpopulation (Th1/Th2)in the peripheral blood of IgAN patients and healthy controls was examined by flow cytometry.The FOXP3 expression was detected through intracellular staining.The correlation of Treg or Th1/Th2 with clinical parameters of IgAN was analyzed by Spearman or Pearson rank correlation test. Results The percentages of Treg and Th2 cells were significantly higher in peripheral blood of IgAN patients compared to that of healthy controls[Treg (2.14±0.82)%vs[1.59±0.53)%,Th2(2.57±0.72)%vs(1.81±1.10)%,all P＜0.05].Th1/Th2 ratio was significantly reduced in IgAN patients(5.75±1.89 vs 12.73±9.79,P＜0.05).The percentage of circulating Treg cells was positively correlated with serunl IgA concentration(r=0.397,P＜0.05),and was negatively correlated with eGFR(r=-0.376,P＜0.05).The percentage of circulating Th2 cells was positively correlated with serum IgA(r=0.468,P＜0.05). Conclusions There is a disorder of T lymphocyte population in the peripheral blood of IgAN patients.The increased Treg and Th2 cells may play an important role in the pathogenesis of IgAN.

Objective To investigate the role of rat organic anion transporter 1 (OAT1, SLC22A6) in the renal cellular uptake of AA Ⅰ and its impact on cellular toxicity. Methods HEK-293 cells were transfeeted with rat OAT1 cDNA or empty vectors. The over-expression of rOAT1 was confirmed by Western blot analysis and its activity was validated by using para-aminohippurate (PAH) as a probe. Cellular apoptosis was examined by flow cytometery using propodium iodode (PI) and annexin V-FITC staining. Results Concentration-and time-dependent intracellular accumulation of AA Ⅰ was observed in rOATl-transfected HEK-293 cells. After treatment with AA Ⅰ at the concentrations of 40 mg/L, 80 mg/L, 120 mg/L and 160 mg/L,respectively, for 45 min, the intracellular concentrations of AA Ⅰ in rOAT1-transfected HEK-293 cells were higher than those in controls (P<0.05). After treatment with AA Ⅰ (120 mg/L for 30 min, 60 min, 90 min and 120 min, respectively, the intracellular concentrations of AA Ⅰ in rOAT1-transfected HEK-293 cells were higher than those in controls (P<0.05). PAH significantly reduced the intracellular accumulations of AA Ⅰ in rOAT1-transfected HEK-293 cells. After treatment with AA Ⅰ at the concentrations of 40 mg/L, 80 mg/L, 120 mg/L and 160 mg/L respectively for 35 min, the intracellular accumulations of AA Ⅰ in rOAT1-transfected HEK-293 cells that treated with PAH were lower than those that were not treated by PAH. Cellular apoptosis and caspase-3 expression in rOAT1-transfeeted HEK-293 cells were significantly up-regnlated as compared to controls (P<0.05). Conclusion rOAT1 is involved in the cellular uptake of AA Ⅰ which leads to increased epithelial apoptosis. Further studies are suggested to investigate the role of human OAT in the disposition of AA and its toxicological consequences.

Objective:Three-dimensional speckle tracking echocardiography (3D-STE) was used to evaluate the left ventricular function in patients with triple vessel coronary artery disease without myocardial infarction.Methods:60 patients with three vessel disease of coronary heart disease without myocardial infarction treated in the Second Affiliated Hospital of Dalian Medical University from June 2018 to December 2020 were selected. They were divided into two groups according to the results of coronary angiography. There were 31 cases in group B, and the stenosis rate of all triple vessel coronary artery was 50%-<75%; 29 cases in group C, the stenosis rate of all triple vessel coronary artery was ≥75%; Thirty healthy subjects were recruited as the group A. We measured left ventricular (LV) end-diastolic and end-systolic volume (LVEDV, LVESV) and LV ejection fraction (LVEF) using real-time dynamic three-dimensional echocardiography. The LV strain parameters were acquired by 3D-STE, including global longitudinal strain (GLS), global area strain, global radial strain (GRS) and global circumferential strain (GCS). The correlation between 3D-STE parameters and N-terminal proBNP (NT-proBNP), left ventricular end-diastolic pressure (LVEDP) were analyzed by Pearson linear correlation analysis.Results:The LVEDV and LVESV in group A and B were significantly lower than those in group C (all P<0.05), and the absolute values of LVEF, GLS, GRS, GCS and GAS were higher than those in group C (all P<0.05). There were no significant difference in LVEDV, LVESV and LVEF between group B and group A (all P>0.05), while the absolute values of GLS, GCS and GAS in group B were significantly lower than those in group A (all P<0.05). The absolute values of GLS, GRS, GCS and GAS in group C were negatively correlated with NT-proBNP and LVEDP (all P<0.05). The absolute value of GLS in group B was negatively correlated with NT-proBNP ( P<0.05), and the absolute value of GLS, GRS, GCS and GAS was negatively correlated with LVEDP (all P<0.05). Conclusions:Our study shows that 3D-STE can evaluate the LV function in patients with triple vessel coronary artery disease without myocardial infarction through multiple strain parameters.

Objective To evaluate the effect of Ulinastatin injection on inflammation, hemodynamics, oxygenation index (PaO₂/FiO₂) and prognosis in patients with septic shock. Methods A retrospective analysis was performed on clinical data of 109 patients with septic shock from January 2017 to December 2019. Patients were divided into observation group (n=73) and routine group (n=54) according to the treatment regimens. The two groups were given anti-shock treatment according to the relevant guidelines, and observation group was additionally given Ulinastatin. The inflammatory factors, hemodynamic parameters and PaO₂/FiO₂ and recovery were compared between the two groups before and after treatment. Results The levels of IL-6, TNF-α and PCT at 7 d of treatment were significantly decreased in the two groups (P<0.05), and the levels of IL-6, TNF-α and PCT of observation group were lower than that of routine group (P<0.05). At 12 h, 24 h and 72 h of treatment, the MAP, CI and PaO₂/FiO₂ were significantly increased in the two groups (P<0.05) while the EVLWI and SVRI were significantly decreased (P<0.05), and the MAP and PaO₂/FiO₂ of observation group at the same time were higher than that of routine group while the EVLWI was lower than that routine group (P<0.05). At 7 d of treatment, the APACHE-II score, mechanical ventilation time and ICU stay time of observation group were lower than that of routine group, and the incidence rates of MODS in the two groups were 4.11% and 14.81% respectively (P<0.05), and the mortality rates were 1.37% and 7.41% respectively (P>0.05). Conclusion Ulinastatin could be beneficial in septic shock, which could reduce inflammatory response, improve hemodynamic parameters and microcirculation perfusion, and put a positive effect on promoting rehabilitation and improving prognosis.

Objective:To explore the effectiveness of application of the internet of things in respiratory rehabilitation nursing for patients with chronic obstructive pulmonary disease (COPD) .Methods:Using the convenient sampling method, a total of 96 patients who were admitted to the Department of Respiration and Critical Care Medicine in the Center of Respiratory Medicine of China-Japan Friendship Hospital from January to December 2019 were selected as research objects. Patients were selected according to their willingness to use the internet of things devices for respiratory rehabilitation. Voluntary users were included in the observation group, while unwilling users were included in the control group, with 48 cases in each group. The control group received routine respiratory rehabilitation nursing, while the observation group received the internet of things scheme on the basis of the control group. The maximum inspiratory pressure (MIP) , activity of daily living (ADL) , rehabilitation exercise compliance and satisfaction were compared between the two groups before and after the intervention.Results:There were significant differences in the MIP and ADL between the two groups before and after the intervention ( P< 0.01) . After the intervention, the MIP, ADL, rehabilitation exercise compliance and satisfaction of patients in the observation group were higher than those in the control group, and the differences were statistically significant ( P<0.05) . Conclusions:The application of the internet of things in respiratory rehabilitation nursing of COPD patients can improve the MIP, ADL, rehabilitation exercise compliance and satisfaction of patients, which is worthy of clinical application and promotion.

Objective:To explore the application effect of respiratory rehabilitation nursing led by respiratory rehabilitation nursing group in patients with acute exacerbation of chronic obstructive pulmonary disease.Methods:Using the convenient sampling method, a total of 124 patients with acute exacerbation of chronic obstructive pulmonary disease who were admitted to Department of Respiratory Medicine of China-Japan Friendship Hospital from June 2018 to June 2020 were selected as the research objects. Among them, 62 cases admitted to the hospital from June 2018 to June 2019 were set as the control group and 62 cases admitted to the hospital from July 2019 to June 2020 were set as the observation group. The control group was given routine nursing, while the observation group was given respiratory rehabilitation nursing by the respiratory rehabilitation nursing group. The differences in body mass index, airflow obstruction, dyspnea, and exercise capacity index (BODE) index, Chinese version of COPD Assessment Test (CAT) scale, activity of daily living (ADL) , self-efficacy and self-management ability were compared between the two groups before and after the intervention.Results:Finally, 58 patients in the control group and 61 patients in the observation group completed the study. After the intervention, the BODE index and CAT score of the observation group were lower than those before the intervention, and the scores of ADL, self-efficacy and self-management ability were increased, and they were all higher than those of the control group, and the differences were statistically significant ( P<0.05) . Conclusions:In the respiratory rehabilitation nursing of patients with acute exacerbation of chronic obstructive pulmonary disease, a special respiratory rehabilitation nursing group can relieve the symptoms of dyspnea and improve the ADL, self-efficacy and self-management ability of patients, which is worthy of promotion.

OBJECTIVE To evaluate the efficacy ，safety，and economy of selexipag in the treatment of pulmonary arterial hypertension（PAH）. METHODS After literature screening ，data extraction and quality evaluation ，the results of included studies were analyzed descriptively by retrieving PubMed ，Embase，Web of Science ，Cochrane Library ，Epistemonikos，SinoMed， CNKI，VIP，Wanfang data ，CBM，official websites of domestic and foreign health technology assessment institutions and relevant database. RESULTS&CONCLUSIONS A total of 7 literature were included ，involving 6 meta-analysis and 1 pharmacoeconomic study. In terms of efficacy ，selexipag could improve patients ’exercise tolerance and hemodynamic indexes ，and reduced the incidence of clinical worsening events and hospitalization . However，intravenous/subcutaneous administration of prostacyclin analog had better effects in improving WHO functional class and reducing all -cause mortality （P＜0.05）. There were no statistically significant differences in the impact on outcome indexes between selexipag and other targeted agents （P＞0.05）. In terms of safety ， the incidence of adverse drug events and drug withdrawal due to adverse reactions was increased by selexipag （P＜0.05），but there were no significant differences between the effects of selexipag and other targeted drugs on adverse events ，serious adverse events and drug withdrawal due to adverse reactions （P＞0.05）. In terms of economy ，based on the background of Canada ’s health system，it was not economical for selexipag to treat PAH ，and there was a lack of economic research based on China ’s national conditions. Further comparative studies on the efficacy and economics of selexipag and prostacyclins in the real world are needed .