Objective Macrophage activation syndrome (MAS) is a severe, potentially life-threatening clinical condition. The clinical features including precipitating events, clinical presentations, treatment strategies, outcome in systemic onset juvenile idiopathic arthritis (So-JIA) children with MAS were reviewed. Perforin A91V gene analysis was also performed. Methods Retrospective review of fourteen MAS cases with So-JIA from 2003 to 2008 from a collected database. Gene-specific polymerase chain reaction ( PCR) primers were used to analyze the perforin A91V gene polymorphism. Results Fourteen patients with age from 4 months to 12 years were considered to have evidence of MAS. Nine of them were boys. The primary diagnosis was systemic onset juvenile idiopathic arthritis. No medication was identified as trigger. Eleven of them had infections prior to MAS. Among them specific infectious agents were identified in four patients. High fever, new onset of hepatosplenomegaly, lymphadenopathy, liver function abnormality, abnormal lipid metabolism and hemophagocytosis were common clinical features. Two cases presented with acute respiratory distress syndrome (ARDS). Multiple organ failure (MOF) occurred in three cases. Three patients died. The variant form (NCBI: SNP rs35947132) of perforin A91V gene was detected in seven systemic onset juvenile idiopathic arthritis compolicated with MAS cases. However no mutation was detected. Clucocorticoid, intravenous immunoglobulin, immunoimpressive therapy were effective treatment of this condition. Plasmapheresis (HP) was successfully used in one case with severe MAS. Conclusions MAS is a rare and potentially fatal complication of childhood rheumatoid diseases such as systemic onset juvenile idiopathic arthritis. In this series, majority of them were male and most of them were preceded by infection. Bone marrow studies support the diagnosis. MOF may be a poor prognostic sign of So-JIA. Aggressive and early therapy is essential. There is no relationship between the variant form (NCBI: SNP rs35947132) of perforin A91V gene and So-JIA with MAS in this small sample's study. More research need to be done by increasing sample's numbers.

BACKGROUND: It has been discovered in experimental researches that qiang chang heji acts on prevention and treatment of brain injuries induced by insufficient blood supply and improvement of blood rhelogical property.OBJECTIVE: To study on the effects of qiang chang heji on peripheral red blood cell in rats with insufficient blood supply to brain so as to probe into its mechauism on improving blood rhelogical property.DESIGN: A randomized controlled trial.SETTING: Central Laboratory of General Hospital of Chengdu Military Area Command of Chinese PLA.MATERIALS: The experiment was performed in Central Laboratory of General Hospital of Chengdu Military Area Command of Chinese PLA from July to September 2003. Totally 50 SD white rats of either sex were employed, weighted varied from 150 g to 170 g.METHODS: The common carotid artery(CA) on the right side was ligatured to induce insufficient blood supply in the brain. The control and qiang chang heji(QCHJ) group were designed for comparison. On the same day of CA ligature, in treatment group, QCHJ of various dosages were applied daily for gastric perfusion; and in the control, physiological saline was applied. Three or seven days later, the parameters of peripheral red blood cell and its rhelogical property were analyzed with equipment.the sham-operation group, mean cell hemoglobin (MCH), haematocrit(HCT) (%) and RBC aggregation index were in the tendency of increase and mean cell hemoglobin concentration (MCHC) (g/L) was increased obviously (375±7 vs 363±13) (P ＜ 0.05) .In the treatment group of 10 g/kg QCHJ, HGB(139.2±12.4 vs 153.6±9.6), MCHC(355±7 vs 375±7) and haematocrit(HCT) (35.4 ±0.9 vs 42.8 ±4.5) were significantly lower the control compared with the sham-operation group, both rigidity index and aggregation index of RBC were in the tendency of further increase. In treatment of QCHJ 10 g/kg, MCHC(354±4 vs 361 ±4), RBC rigidity index (1.02±0.35 vs 4.54±3.04) and aggregation index (2.76±0.66 vs 4.67 ± 1.21 ) were significantly lower than the control( P ＜ 0.05).CONCLUSION: Treatment with QCHJ can prevent changes in peripheral red blood cell in rats with insufficient blood supply in the brain and reduce MCHC and RBC rigidity index and aggregation index so as to improve RBC deformability and rhelogical property.

BACKGROUND: It has been shown that Qiang Chang Heji (QCHJ) hadsome protective effects in rats with experimental brain injuries and couldimprove some hemorheological indices.OBJECTIVE: To observe effects of QCHJ on peripheral platelet in rats with experimental right brain insufficiency of blood supply.DESIGN: A randomized and controlled experiment.SETTING: Central Laboratory, General Hospital of Chengdu Military Area Command of Chinese PLA.MATERIALS: Totally 60 SD white rats (either sex, werghing from 150 g to 170 g) were used. They were divided randomly into 3 groups: sham-operation group (n=8), control group (n=18) and treatment group (n=34).QCHJ:Chinese herbal mixture, equal dosage of both Rhizoma seu Radix Notopterygii and Rhizoma Acori Graminei were decocted twice, each for 30minutes with mild fire after boiling. The two decoctions were mixed and concentrated to 1g raw herb per milliliter.METHODS: The experiment was carried out in Central Laboratory, General Hospital of Chengdu Military Area Command of Chinese PLA from July to September 2004. The right carotid artery (CA) of rats were separated,ligaturewas done in control and treatment groups ;but no ligature in sham operation group. Medication (po. by gastric perfusion) was given since the day of operation. In treatment group, 10 rats took QCHJ 10 g/kg per day for3 days; and others took 3.3, 6.7, 10 g/kg per day for 7 days respectively, (8 rats for each dose). In control group, equal volume physiological saline was given daily for 3 or 7 days. In sham operation group, equal volume physiological saline was given daily for 3 days. On the 3rd and 7th days after operation, platelet count (Plt), mean platelet volume (MPV), platelet distribution width (PDW) and platelet-large cell ratio (P-LCR) of platelet were determined with a Bakeman Blood Cell Auto-analyzer. t test was used to compare the difference.MAIN OUTCOME MEASURES:Effects of QCHJ on peripheral platelet in rats with brain insufficiency of blood in brain.RESULTS: ①Day 3 after operation: PLt were similar between control group and sham-operation group (P ＞ 0.05); MPV, PDW and P-LCR were higher in control group than that in sham operation group (P ＜ 0.01); in QCHJ treated group (10 g/kg per day), MPV, PDW, and P-LCR were significantly lower than that in control group (P ＜ 0.01), and they were similar to that in sham operation group. ② Day 7 after operation: MPV, PDW, and P-LCR in control group were similar to that in sham operation group. In addition, P-LCR in control and QCHJ treatment group (3.3, 6.7, 10.0 g/kg per day) were lower than that in control group on the 3rd day of operation (P ＜ 0.01).CONCLUSION: Experimental brain injury induced by block blood supply may result in peripheral platelet abnormities. QCHJ has been shown to improve some of the abnormities.

BACKGROUND: Cells in contact with nanomaterials can induce oxidative stress, allergic reactions, and then produce cytotoxicity and genotoxicity. Therefore, studies on nano toxicology have attracted more and more attention.OBJECTIVE: To comparatively evaluate the cytocompatibility of Pluronic (P85, F127, F87) tri-block copolymer nanoparticles modified with folic acid (FA) and polylactic acid (PLA).METHODS: Pluronic (P85, F127, F87) tri-block copolymer nanoparticles were modified with FA and PLA to synthesize a variety of amphiphilic block copolymers, including PLA-P85-PLA, FA-P85-PLA, PLA-F127-PLA, FA-F127-PLA,PLA-F87-PLA and FA-F87-PLA. The cytotoxicity of these synthesized nanoparticles was analyzed by cell morphology,cell metabolic activity and cell membrane effects in HepG-2 cells.RESULTS AND CONCLUSION: The relative growth rate of HepG-2 cells had no significant differences under 24-hour induction of various concentrations (5, 10, 20, 50, 100 mg/L) of unmodified P85, F127, and F87 nanoparticles (P > 0.05).The growth and proliferation of cells under the low concentrations (5, 10, 20, 50 mg/L) were enhanced. P85 NPs and F87 NPs could significantly inhibit cell viability at dose of 400 mg/L. In contrast, there were no significant differences towards P85, F127 and F87 nanoparticles (5, 10, 20, 50, 100, 200, 400 mg/L) modified with FA and PLA when compared with the control group (P > 0.05). These findings indicate that the modification of FA and PLA can improve the cytocompatibility of Pluronic (P85, F127, F87) tri-block copolymers, and therefore, PLA-Pluronic-PLA and FA-Pluronic-PLA nanoparticles are both good candidates for drug vectors.

Objective:To investigate the therapeutic efficacy of Golimumab in the treatment of children with refractory juvenile dermatomyositis(JDM).Methods:The clinical data of a child diagnosed with JDM in the Department of Allergy, Immunology and Rheumatology of Guangzhou Women and Children′s Medical Center in February 2019 were collected.The treatment effect was studied and literature review was conducted.Results:The patient was a 7-year-old boy with subacute onset of the disease.The illness protracted, and main manifestations included skin rashes, limb weakness, and swallowing dysfunction.Physical examination showed heliotropic rashes, Gottron papules, positive Gower, proximal limb muscle strength grade Ⅲ-Ⅳ, distal limb muscle strength grade Ⅳ, and a choking cough when swallowing fluid food.Laboratory tests revealed alanine aminotransferase (ALT) of 36 U/L, aspartate aminotransferase (AST) of 115 U/L, alkaline phosphatase of 69 U/L, lactate dehydrogenase of 941 U/L, creatine kinase of 974 U/L, hypersensitive C-reactive protein of 26 mg/L and an erythrocyte sedimentation rate (ESR) of 52 mm/1 h. Antinuclear antibody spectra were negative.Electromyography suggested myogenic damage.Thigh magnetic resonance imaging indicated diffuse abnormal signal shadows in the subcutaneous fat, muscles and muscle spaces of both hips, thighs and knee joints.The child was diagnosed with JDM, and given standardized treatment of Methylprednisolone, intravenous immunoglobulin, Methotrexate and Hydroxychloroquine sulfate.However, after the treatment, the facial rashes were still red, proximal limb muscle strength and swallowing dysfunction did not improve, the choking cough symptom still existed, and a Cushing face appeared.Recheck results showed ALT of 24 U/L, AST of 32 U/L, alkaline phosphatase of 56 U/L, lactate dehydrogenase of 216 U/L, creatine kinase of 527 U/L, hypersensitive C-reactive protein of 8 mg/L and an ESR of 15 mm/1 h. Refractory JDM was considered.After negotiating with the patient′s family members, they agreed to treat the patient with Golimumab 50 mg by subcutaneous injection once a month.Then tapered prednisone gradually, stopped Hydroxychloroquine sulfate tablets and continued to give the patient oral Methotrexate.After two doses of Golimumab 50 mg, proximal limb muscle strength and swallowing function improved markedly.After the third subcutaneous injection of Golimumab, proximal limb muscle strength improved to grade Ⅳ-Ⅴ, and he was able to go up and down stairs, squat and stand up after squatting.Besides, dysphagia and the choking cough disappeared, and skin rashes improved.Recheck results suggested a normal ESR and creatine kinase levels.Magnetic resonance imaging of thighs indicated no muscle inflammation.Conclusions:Golimumab works well in the treatment of refractory JDM and can effectively improve muscle strength.Therefore, it can be used as a treatment option for refractory JDM.

OBJECTIVETo show the changes of heat shock protein 70(HSP70) expression at cellular level in different heat exposure stages and the significance of HSP70 expression in heat exposure organism.

METHODThe heat exposure model was established in rats with artificial hot climatic chamber[34 +/- 1) degree C, RH 60%]. SD rats were randomly divided into control groups(C) and heat exposure groups(A), and into subgroups including 2, 7, 14, 28 d stages from each one of the groups. The immuno-histochemistry was used to detect HSP70 expression in rat liver and cardiac muscle, and photography analytic software was used to analyze HSP70 expression in liver and cardiac cells.

RESULTSThe expression intensity of HSP70 in heat exposure groups(gray values of liver were 137.0 +/- 5.1, 137.0 +/- 5.2, 137.8 +/- 7.1, 139.2 +/- 5.2 respectively; of cardiac muscle 156.1 +/- 4.4, 155.1 +/- 6.2, 155.4 +/- 4.5, 156.2 +/- 5.1 respectively) was stronger than that in control groups(P < 0.01 or P < 0.05) during all stages of the heat exposure; There was no significant difference in expression intensity of HSP70 among various stages of heat exposure; after 2 d of heat exposure, HSP70 expression in cell nuclei of the liver and cardiac muscle cells was stronger than that in cytoplasm in heat exposure group; HSP70 expression in Kupffer's cells of liver was also stronger than that of control(P < 0.05), but not on 7, 14 and 28 d; the activities of alanine aminotransferase(ALT), aspartate aminotransferase(AST), creatine kinase(CK) and hydroxybutyrate dehydrogenase(HBD) showed an increase on 2 d and 28 d of heat exposure.

CONCLUSIONThe vital organs would be damaged on 2 d of heat exposure. High expression of HSP70 at this stage may be a marker of cell damage; Increased HSP70 expression on 7-14 d of heat exposure may play an important role in adaptation to heat, while long term(28 d) heat exposure, the protection of HSP70 from tissue damage may not be enough.

Adaptation, Physiological ; Animals ; HSP70 Heat-Shock Proteins ; biosynthesis ; Heat Stress Disorders ; metabolism ; Liver ; metabolism ; Myocytes, Cardiac ; metabolism ; Rats ; Rats, Sprague-Dawley ; Time Factors

Objective To evaluate the effect of propofol on the expression of programmed death-ligand-1 (PD-L1) in pancreatic cancer cells and the relationship with NMDA/Ca2+/calmodulin-dependent protein kinase Ⅱ (CaMK Ⅱ)/hypoxia-inducible factor-1α (HIF-1α) pathway.Methods Human pancreatic cancer cells were divided into 5 groups (n=16 each) by a simple random sampling method:control group (group C),propofol group (group P),KN93 (CaMK Ⅱ inhibitor) group,MK801 (NMDA receptor antagonist) group and propofol plus rapastinel (NMDA receptor agonist) group (group PR).Cells were cultured in DMEM supplemented with 10％ fetal bovine serum in group C.Cells were incubated for 8 h with 50 μmol/L propofol in group P.Cells were incubated for 8 h with 10 μmol/L KN93 in group KN93.Cells were incubated for 8 h with 500 μmol/L MK801 in group MK801.Cells were incubated for 8 h with 50 μmol/L propofol and 20 μmol/L rapastinel in group PR.After the end of treatment in each group,the cell viability was measured using CCK8 assay,the expression of PD-L1,HIF-1α,CaMK Ⅱ and phosphorylated CaMK Ⅱ (p-CaMK Ⅱ) was detected by Western blot,and intracellular calcium concentrations were determined by Fluo3/AM probe.Results Compared with group C,the cell viability was significantly decreased,the expression of PD-L1,HIF-1α and p-CaMK Ⅱ was down-regulated,and intracellular calcium concentrations were decreased in P,KN93 and MK801 groups (P＜0.05),and no significant change was found in group PR (P＞0.05).Compared with group P,the cell viability was significantly enhanced,the expression of PD-L1,HIF-1α and p-CaMK Ⅱ was up-regulated,and intracellular calcium concentrations were increased in group PR (P＜0.05).Conclusion The mechanism by which propofol inhibits the malignant potential of pancreatic cancer cells may be related to inhibiting NMDA/CaMK Ⅱ/HIF-1α pathway and down-regulating PD-L1 expression.

Ischemic stroke can cause severe brain damage in the short term after an ischemic attack. ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13) is a von Willbrand factor lyase that can affect both thrombosis and inflammation. It plays an important role in the occurrence, development, and outcome of ischemic stroke. In recent years, research on ADAMTS13 and ischemic stroke has gradually increased and there are some new developments. This article reviews the role of ADAMTS13 in the onset, outcome, and treatment of ischemic stroke.

Objective:To explore the surgical technique and the clinical efficacy of the lateral-rectus approach with traction reduction by the modified Starr pelvic reduction frame for Tile C pelvic nonunions and malunions.Methods:Data of 7 patients with Tile C pelvic nonunions and malunions from June 2017 to June 2018 who were treated via the lateral-rectus approach combined with traction reduction by the modified Starr pelvic reduction frame were retrospectively analyzed. There were 5 males and 2 females, aged from 22 to 53 (mean, 40 years). The injury mechanism included 3 cases of car accident injury, 3 of falling injury and 1 of crushing injury. The reasons for retreatment were pain in 7 cases, inability to walk and sit in 3, unequal length of lower limbs and lameness in 3. According to Tile classification, there were 4 cases of C1 type, 2 of C2 type and 1 of C3 type. A complete pelvic model with equal size as the patient’s pelvis was 3D-printed out according to three-dimensional reconstruction CT. The osteotomy or release site was designed, and the preoperative plan was detailed. Expose and release via the lateral rectus approach combined with traction reduction was conducted using the modified Starr pelvic reduction frame. Operative time, intraoperative blood loss and postoperative complications were collected. Visual analogue scale (VAS) at 6 months after surgery were recorded. Majeed score was used to evaluate the clinical efficacy. The quality of fracture reduction was evaluated by the Mears-Velyvis radiological evaluation criterion at the latest follow-up.Results:The operation time was 140-280 min, with an average of 190 min. The intraoperative blood loss was 700-2,800 ml, with an average of 1,250 ml. In 6 cases, the final fixation was performed at one time, while 1 case of Tile C3 type was performed in two stages. All patients were followed up for 10-22 months, and all the fractures healed. The mean time of bony union was 8 weeks (range, 6-12 weeks). The VAS of the 7 patients was improved from an average of 6.4 points to an average of 0.7 points during 6 months postoperative follow-up. The Majeed clinical efficacy score of the latest follow-up was improved from the average 60 points preoperative to 85 points postoperative. According to the Mears-Velyvis radiological evaluation criterion, the satisfaction rate reached 85.7%(6/7). After operation, 1 case occurred obturator nerve injury who recovered within 3 months, and 1 case remained limb shortening deformity of 1 cm. There was no fixation failure.Conclusion:The anterior osteotomy via the lateral-rectus approach can fully cut off nonunions and malunions of the pelvis, effectively release the soft tissues around the osteotomy site, with minimal surgical trauma and low risks of neurovascular injuries. Combined the modified Starr pelvic reduction frame, it can effectively correct pelvic deformities, lower limb rotation and unequal length deformities to achieve the expected effect of surgery.

Objective:To establish the quality evaluation method of Prunellae spica dispensing granules based on three quality indexes of standard decoction. Methods:Fourteen batches of Prunellae spica were collected from different habitats. According to technical requirements, fourteen batches of Prunellae spica standard decoction and three batches of formula granules were prepared and the paste-forming rates were calculated. The fingerprints of Prunellae spica standard decoction and formula granules were established by Ultra High Performance Liquid Chromatography (UPLC). The similarity values of fingerprints between dispensing granules and standard decoction were calculated. The content and transferring rate of Rosmarinic acid were determined and calculated. Results:The average paste-forming rate of Prunellae spica was (12.59±2.32)%. The paste-forming rates of the three batches were 11.14%, 10.78% and 10.39% respectively. The average content of Rosmarinic acid in standard decoction was (18.99±9.74)mg/g. The average transferring rate was (60.58±7.87)%. The contents of three batches were 7.40 mg/g, 7.49 mg/g and 7.09 mg/g. The transferring rates were 52.06%, 50.10% and 50.40% respectively. Nine common fingerprint peaks were identified in the fingerprints of standard decoction and formula granules, two of which were identified as Rosmarinic acid and Caffeic acid by comparison of reference substance. The fingerprints similarity of Prunellae spica dispensing granules and standard decoction were 0.954, 0.973 and 0.952, respectively. Conclusions:The quality indexes of three batches of formulation granules are consistent with standard decoction. This method could provide reference for the establishment of quality standard of Prunellae spica dispensing granules.