Objective:To compare the similarities and differences of macrophage activation syndrome（MAS）combined with systemic juvenile idiopathic arthritis（sJIA）versus with juvenile onset systemic lupus erythematosus（JSLE）.Methods:The clinical data of 48 children with MAS admitted to the Department of Nephrology and Immunology in Children's Hospital of Hebei Province from May 2015 to January 2023 were retrospectively analyzed. The patients were divided into sJIA-MAS and JSLE-MAS group，and the clinical manifestations，laboratory indicators and treatment of the two groups were compared.Results:Among the 48 children（14 males and 34 females）with MAS，the average age of onset was 9.5（3.0，11.8）years. There were 28 cases（11males and 17 females）of sJIA-MAS and 20 cases（3 males and 17 females）of JSLE- MAS. All the 48 children with MAS had fever and hyperferinemia，and the fever with sJIA-MAS was mostly continued fever or remittent fever. Respiratory tract infection was the most common trigger in sJIA-MAS［15 cases（53.6%）］，and disease activity was the most common trigger in JSLE-MAS［13 cases（65.0%）］.Additionally，viral infections（EB virus and cytomegalovirus）were also one of the triggers in MAS［sJIA：7 cases（25%），JSLE：4 cases（20%）］.Compared with JSLE-MAS，the number of days with fever［15.0（12.0，21.0）days vs. 6.0（4.0，9.5）days， Z=-3.812， P=0.001］and the length of hospital stay［29.0（26.3，39.8）days vs.26.0（19.3，30.8）days， Z=-1.958， P=0.049］were longer in sJIA. Compared with JSLE-MAS，ALT［（685.32±561.67）U/L vs.（139.61±124.44）U/L， t=4.973， P=0.001］，AST［784.00（235.25，1 251.25）U/L vs.189.50（53.25，374.08）U/L， Z=-3.283， P=0.001］，CRP［11.48（3.56，28.89）mg/L vs.1.91（0.53，8.98）mg/L， Z=-3.200， P=0.001］，ferritin［32 167.0（12 384.8，65 963.8）μg/L vs.2 003.5（922.5，11 430.0）μg/L， Z=-4.130， P=0.001］，ferritin max/ESR min［1 353.35（355.75，4 342.53）vs.91.92（34.94，291.53）， Z=-4.120， P=0.001］were higher in sJIA.The decrease of CRP was greater in sJIA［80.04（45.64，143.71）mg/L vs.10.20（6.27，25.64）mg/L， Z=-4.433， P=0.001］.Compared with sJIA-MAS，peripheral white blood cell counting［4.05（2.90，7.73）×10 9/L vs.1.56（1.15，3.47）×10 9/L， Z=-3.577， P=0.001］and platelet counting［（162.68±92.19）×10 9/L vs.（110.10±72.99）×10 9/L， t=2.118， P=0.040］were lower in JSLE-MAS. Kidney involvement was more common in JSLE-MAS［10 cases（50%）vs.0 cases（0%）， χ 2=17.684， P=0.001］.There was no significant difference in the incidence of sJIA-MAS and JSLE-MAS meeting the criteria of hemophagocytic lymphohistiocytosis［6 cases（21.4%）vs.5 cases（25.0%）， χ 2=0.084， P=0.772］. Conclusion:Compared with JSLE-MAS，sJIA-MAS is more dangerous and difficult to control，while JSLE-MAS involves more organs，among which the blood system and kidney are more common.

Objective To explore the application effect of advanced lung rehabilitation programs in patients un-dergoing high flow nasal cannula oxygen therapy(HFNC).Methods Convenience sampling method was used to se-lect 40 HFNC patients admitted to the respiratory department,ICU,coronary heart disease monitoring unit,and other departments of a tertiary A comprehensive hospital in Tianjin from January to June 2023 as the experimental group,and 40 HFNC patients admitted from June to December 2022 as the control group.Both groups of patients received HFNC treatment during hospitalization and continued at home,while the experimental group adopts the information-motivation-behavioral skills(IMB)model as the theoretical basis,implements advanced pulmonary rehabili-tation during the stable condition period,discharge preparation period,and home rehabilitation period,and imple-ments compliance management of home pulmonary rehabilitation through cloud follow-up,and the control group re-ceived routine lung rehabilitation and home follow-up.The degree of respiratory distress,6-minute walking distance,exercise self-efficacy,quality of life were compared between the 2 groups before intervention,2 months and 6 months after intervention,and compliance with home pulmonary rehabilitation was compared between the 2 groups 6 months after intervention.Results The repeated measures analysis of variance showed that there was an interaction effect between time and the 2 groups in terms of difficulty in breathing,6-minute walking distance,exercise self-ef-ficacy,and quality of life(P＜0.05).Simple effect analysis showed that after 2 and 6 months of intervention,the ex-perimental group performed better than the control group in the respiratory distress,6-minute walking distance,exer-cise self-efficacy,and quality of life(P＜0.05).Within 6 months of intervention,the compliance of home pulmonary rehabilitation in the experimental group was better than that in the control group,and the difference was statistically significant(P＜0.05).Conclusion The application of advanced pulmonary rehabilitation programme based on IMB can improve respiratory symptoms and improve exercise self-efficacy and adherence to pulmonary rehabilitation,en-hance activity endurance and improve quality of life.

Objective:To investigate the role and mechanism of retinal Sigma-1 receptor antagonist N, N-diethyl-2-[4-methoxy-3-(2-phenylethoxy)phenyl]ethanaminehydrochloride (NE-100) in the formation of form deprivation myopia (FDM) in guinea pigs.Methods:Eighty-five 21-day-old guinea pigs were selected, and 36 of them were randomly divided into normal control group, occluded 14-day group and occluded 11-day group, with 12 in each group.The right eyes of guinea pigs in the occluded 14-day group were covered with translucent eye shield for consecutive 14 days, and guinea pigs in the occluded 11-day group were treated in the same way for consecutive 11 days plus 3 days without cover, and guinea pigs in the normal control group were not covered.The other 49 guinea pigs were randomly divided into FDM group ( n=10), FDM+ NE-100 6 μg group ( n=12), FDM+ NE-100 60 μg group ( n=10), FDM+ NE-100 600 μg group ( n=9), and FDM+ saline group ( n=8). The right eyes in each group received 100 μl peribulbar injection of NE-100 6 μg, 60 μg and 600 μg or saline once a day according to grouping.Ocular refraction and axial dimensions were measured using eccentric infrared photorefractor and A-scan ultrasonography, respectively.Corneal curvature was measured with keratometer.Immunohistochemical staining and Western blot were used to detect the expression levels of Sigma-1 receptor protein, and retinal dopamine content was evaluated by high-performance liquid chromatography with electrochemical detection.This study was approved by an Ethics Committee of the Department of Laboratory Animal Science of Central South University (No.2020sydw0084). The use and care of experimental animals followed the Regulations for the Administration of Affairs Concerning Experimental Animals in China. Results:There were statistical significant differences in diopter and axial length among the normal control group, occluded 14-day group and occluded 11-day group ( F=147.81, 160.10; both at P<0.01). Compared with the normal control group, the relative myopia was the deepest and the axial length was the longest in the occluded 14-day group, then the occluded 11-day group, showing significant differences between them (all at P<0.05). In the normal control group, Sigma-1 protein was mainly expressed in retinal ganglion cells (RGCs), photoreceptor inner segment and the inner nuclear layer.In the occluded 14-day group, Sigma-1 protein staining was enhanced in RGCs and photoreceptor inner segment.Sigma-1 staining positive cells in the inner nuclear layer were increased significantly and were also seen in the inner and outer plexiform layers, especially in Müller cells, in which the expression levels of Sigma-1 receptor protein were significantly increased.Compared with the occluded 14-day group, the Sigma-1 receptor protein expression levels in the retina of the occluded 11-day group was significantly decreased ( P<0.01). The diopters of guinea pigs in the FDM+ NE-100 6 μg, 60 μg and 600 μg groups were lower than those in the FDM group, and the diopters of FDM+ NE-100 60 μg and 600 μg guinea pigs were lower than those in the FDM+ NE-100 6 μg group, and the differences were statistically significant (all at P<0.05). The dopamine content in the retina of the FDM+ NE-100 60 μg group was (0.74±0.09) ng/mg, which was significantly higher than (0.57±0.10) ng/mg in the FDM group, with a significant difference between them ( t=15.18, P<0.01). Conclusions:Sigma-1 receptor antagonist inhibits FDM formation, which may be associated with the elevation of dopamine content in retina.

Objective To investigate the expression of elongation of very long-chain fatty acids family member 6 (ELOVL6) in high-grade serous ovarian carcinoma (HSOC), and explore the correlation between its expression and clinical prognosis in these patients. Methods The expression of ELOVL6 at mRNA and protein levels were respectively detected by reverse transcription (RT)-PCR and immune histochemistry method in 12 cases with normal ovarian tissues and 172 cases with HSOC from primary tumor site, forty of which had paired peritoneal metastatic tissues. Results (1) The results tested by RT-PCR showed that ELOVL6 expression in normal ovarian tissue was 4.8±1.1, while 1.2±0.7 in primary tumors and 1.8 ± 0.9 peritoneal metastatic sites in HSOC. Compared with normal ovarian tissue, the level of ELOVL6 mRNA was significantly lower in HSOC (P<0.05). There was no significant difference between primary and peritoneal metastatic sites in HSOC (P=0.610). It was shown that ELOVL6 protein localized in cytoplasm of ovarian cancer cell by immunostaining assay. (2) ELOVL6 expression was observed in all normal ovarian tissue, 70.2%of G1-G2 and 48.8%of G3 HSOC (P<0.05). ELOVL6 expression in drug-resistant group were significantly lower than that in non-resistant group (39.1% vs 65.0%, P<0.01). The median disease-free survival was 41 months in the ELOVL6-positive group and 39 months in ELOVL6-negative group (P>0.05). The total median survival was 52 months in ELOVL6-positive group and 44 months in ELOVL6-negative group (P>0.05). Conclusion Low expression of ELOVL6 may correlate with the poor differentiation and drug resistance in HSOC.

Objective To analyze the relationship between clinical and pathological features in children with Henoche-Sch?nelin purpura nephritis (HSPN). Methods Clinical and pathological data of 89 children with HSPN from April 2009 to April 2013 in our hospital were retrospectively analyzed. Results A majority of patients (94.83%) suffered nephritis within two month after purpura. Clinically, hematuria and proteinuria (41.6%), nephrotic syndrome (27.0%), and isolated protein-uria (21.3%) are the most common type in children with HSPN. The majority of pathological changes of these three types HSPN are gradeⅢ, which accounts for 67.6%, 70.8%, and 73.7%, respectively. The clinical manifestations are closely associated with pathologic classiifcation (H=26.88, P=0.000). IgA plus IgM deposit was the most common type (40.45%) in histological and immunopathologic changes.There was no statistical difference between the pathologic classification and immunopathologic types. Gastrointestinal bleeding was associated with pathologic classiifcation. Conclusions The pathological changes of HPSN patients are mainly of gradesⅡandⅢ, and the clinical manifestations, gastrointestinal bleeding in particular, are closely associated with pathological classiifcation.

BACKGROUNDAberrant epithelial repair has been observed in chronic rhinosinusitis (CRS) patients; however, the mechanism of epithelial cell repair regulation is unclear. Epidermal growth factor (EGF) plays an important role in regulating epithelial cell repair in lower airway and may be a critical factor in the remodeling processes of CRS. The objective of our research is to evaluate the differences between CRS and normal subjects and between chronic rhinosinusitis without nasal polys (CRSsNP) and chronic rhinosinusitis with nasal polys (CRSwNP) in the regulation of EGF pathways and the regulating proliferative position of classic Ras/Raf/MEK/ERK pathways.

METHODSWe evaluated the proliferation rates of ethmoidal mucosal cells before and after stimulation with EGF, epidermal growth factor receptor (EGFR) kinase inhibitor AG1478, and extracellular signal-regulated kinase 1/2 (ERK1/2) inhibitor PD98059 using MTT assays. We also analyzed the sinonasal epithelial cells collected from control subjects and patients with CRS subtypes CRSsNP and CRSwNP for the expression of ERK1/2, phosphorylated ERK1/2, P21, P15, and P27 using western blotting analyses.

RESULTSThe proliferation rates of sinonasal epithelial cells before and after EGF stimulation were lower in CRS patients than in the controls. AG1478 or PD98059 inhibitor treatment of control epithelial cells did not result in a significant difference in proliferation. Although, AG1478 and PD98059 inhibited the proliferation of CRS cells, the degree of proliferation inhibition was markedly different in CRSsNP. AG1478 suppressed the proliferation of CRSwNP epithelial cells, whereas PD98059 had no effect. The ratio of ERK1/2 phosphorylation in CRS cells was lower than that of the control cells. Cyclin-dependent kinase inhibitors were highly expressed in CRS cells compared with that of control cells. ERK1/2 and P27 showed differential expression in CRSsNP and CRSwNP.

CONCLUSIONSDifferences existed in EGF pathways in CRS patients and normal subjects as well as in CRSsNP and CRSwNP. Classical Ras/Raf/MEK/ERK pathway may assume absolute superiority in control cells. Ras/Raf/MEK/ERK classical pathway and other pathways might be active at the same time to stimulate epithelial cell proliferation in CRSsNP. The function of Ras/Raf/MEK/ERK classical pathway was weaker in CRSwNP than in CRSsNP and when the classical pathway was blocked in CRSwNP, some other pathway could have completely compensated the proliferation induced by the Ras/Raf/MEK/ERK pathway.

Adult ; Aged ; Cell Proliferation ; drug effects ; Cells, Cultured ; Epidermal Growth Factor ; pharmacology ; Epithelial Cells ; cytology ; drug effects ; Female ; Humans ; Male ; Middle Aged ; Nasal Mucosa ; cytology ; Sinusitis ; metabolism

Objectives To investigate the clinical effect of hemoperfusion in severe juvenile dermatomyositis. Meth-ods Two patients with severe juvenile dermatomyositis, who accepted hemoperfusion treatment, were retrospectively analyzed. Results In these 2 patients, the hemoperfusion process were smooth. The muscle pain was reduced and the muscle enzymes were decreased. After hemoperfusion, one patient refused to continue treatment and the disease recurrent. The other patient con-tinued to accept the immunosuppressive therapy and eventually improved. Conclusions For juvenile dermatomyositis, hemo-perfusion was a safe and effective auxiliary treatment. It could be applied when the general treatment was invalid.

Objective To investigate the chanegs and correla iton between IL-17 and MMP-9 in c hildren of henoch-schonlein purpura,and to explore the pathogenesis of teh IL-17 and MMP-9 in henoch-schonlein pur-pura /henoch-schonlein purpura nephritis.Methods The level of serum IL-17 andMMP-9 in 74 henoch-schon-lein purpura childr en in initial stage and 30 cases of healthy children( control group) were detetced by ELISA. Results (1) The level of plasma IL-17 was significantly higher in children of henoch-schonlein purpura thna tha t foh ealthy control group (86.59 ±35.50) fg/L vs.(62.38 ±14.65)fg/L,P<0.01].(2) The level of plasma MMP-9 was significantly higher in children of henoch-schonlein pupru ar than that o fhealt hy control group [(201.82 ±105.87) fg/L vs(89.27 ±27.99)fg/L,P <0.01].(3) Henoch-schonlein purpura children were followed up for6 months,dependin g on whether the kidney was involved,they were devided into non-he-noch-schonlein purpura nephritsi grou p nad henoch-s chonlein purpura nephritis group.In initial phase of HSP,the level of plasma IL-17 was significant lyhigher in henoch-schonlein purpura nephritis group than non-henoch-schonlein purpura nephrits group [(101.67 ±39.55) fg/L vs.(101.67 ±39.55)fg/L,P<0.05].The level of p lasma MMP-9 was significantly higher in henoch-schonlein purpura nephritsi group than no n-henoc h-schon-lein purpura nephritis group [(249.63 ±97.57) Pg/L vs.(185.30 ±104.39) pg/L,P<0.05 ].(4) IL-17 and MMP-9 correlation aanlysis didn't show sign ificant correlation(r=0.184,P>0.05).Conclusion IL -17 and MMP-9 are involved in the pathogenesis of henohc-schonlein purpura/henoch-schonlein pur pura nephritis.

ObjectiveTo explore the points of nursing cooperation during hand-assisted thoracoscopic surgery for simultaneous operation of bilateral lung metastasectomy.Methods The preoperative preparation,nursing cooperation during the surgery,the cleaning and maintenance of surgical instruments and equipment after operation were retrospectively summarized in 72 patients who underwent hand-assisted thoracoscopic surgery for simultaneous treatment of bilateral pulmonary metastases. ResultsAll the procedures of surgery were smooth.No patients were converted to thoracotomy or died of surgery. Conclusions It is an important challenge for nursing quality in the operation room and the cleaning and maintenance of surgical instruments and equipment in carrying out hand-assisted thoracoscopic surgery.The skilled nursing cooperation and standardized operating procedures could improve the quality of intraoperative nursing cooperation.It could ensure the smooth process of simultaneous operation of bilateral lung metastasectomy.

Objective: To evaluate the clinical effect of auricular sticking therapy in treating vascular headache. Methods: Fifty patients with vascular headache were randomized into two groups, 25 cases in each. The treatment group received auricular sticking therapy with magnetic pearls, and the control group was treated by Flunarizine Hydrochloride, 30 d as a treatment session, and the results were measured after one treatment session. Result: The total effective rate was 92.0% in the treatment group while 60.0% in the control group, and the difference was significant (P<0.01). A marked decrease of blood pressure was detected in the treatment group (P<0.05). The mean velocity in middle cerebral artery and anterior cerebral artery both changed significantly (P<0.01). Conclusion: Auricular sticking therapy can significantly improve the condition of vascular headache, as well as reduce blood pressure and help cerebral blood flow.