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 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.

OBJECTIVE： The preventive and therapeutic effects of a compound traditional Chinese medicine preparation， Yingganfukang(YGFK)， on mice experimental liver fibrosis were studied METHODS： The acute liver damage and fibrosis rat models were established by giving single or multi－ dose of CCl4 orally and the liver dysimmunity model was established by giving BCG and LPS intravenously Rats were divided into YGFK group and control group The serum ALT， AST and ALP of the mice were examined The liver tissues taken from the liver fibrosis mice were committed to pathological examination RES_ ULTS： The serum ALT， ALP of the YGFK(2g/kg· d) group and control group were （ 6 153± 3 491） IU/L、 （ 202± 24） IU/L and （ 9 275± 2 744） IU/L、 （ 421± 67） IU/L， respectively in the acute liver damage mice with significant differences

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.

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 analyze the relationship between Notch signaling pathway and levels of lymphocytes and cytokines in children with juvenile idiopathic arthritis (JIA),and to explore its role in the pathogenesis of JIA.Methods Thirty-five pediatric patients with JIA [males 20 cases,females 15 cases;aged (6.5 ±4.0) years old,(0.83-15.00 years old)] and 15 healthy children [males 6 cases,females 9 cases;aged (5.0 ± 2.9) years old,(1.0-11.0 years old)] from November 2015 to February 2016 in Guangzhou Women and Children's Medical Center were included in the study.The JIA group were divided into the systemonset JIA(So-JIA) group (22 cases) and psoriatic JIA(p-JIA) group (13 cases,polyarthritis 7 cases and oligoarthritis 6 cases).The expressions of Notch signaling's receptor,ligand and target gene mRNA in peripheral blood monouclear cells (PBMC) from the JIA group and the control group were determined by quantitative real-time PCR.The levels of cytokines interleukin (IL)-1 β,IL-10,IL-6,IL-17,IL-4 and transforming growth factor-β (TGF-β) were detected by enzyme-linked immunosorbent assay.Results Compared with the healthy control group,the Notch2 receptors (1.6 ± 3.2 vs.0.4 ± 0.3) expression level,Jagged1 ligand (44.0 ± 79.0 vs.11.3 ± 1.2) expression levels and the levels of target gene HES1(0.4 ±0.3 vs.0.1 ± 0.1) mRNA in the JIA group showed a significant increase,and the differences were all statistically significant (all P ＜0.05).Compared with the healthy control group,the JIA group showed an increased level of IL-1β [(182.22 ± 309.13) ng/L vs.(54.71 ± 20.33) ng/L],IL-10 [(32.99 ± 34.28) ng/L vs.(22.68 ±4.56) ng/L],IL-6 [(100.48 ±305.57) ng/L vs.(13.98 ±2.78) ng/L],IL-17 [(9.11 ± 17.57) ng/L vs.(2.42 ±0.29) ng/L] and TGF-β [(14.37 ±9.33) ng/L vs.(5.49 ±4.49) ng/L],and there were statistically significant differences (all P ＜ 0.05).The expression level of HES1 mRNA was positively correlated with STAT3 mRNA in the So-JIA group (r =0.573,P ＜0.05).The expression level of HES1 mRNA was positively correlated with CD3 + T(r =0.528),CD19 + B (r =0.480),CD3 + CD4 + TH(r =0.457) and CD16 + CD56 + NK (r =0.598) cell absolute count in the So-JIA group (all P ＜0.05).The expression level of HES1 mRNA was positively correlated with CD3 + T cell absolute count in the p-JIA group (r =0.577,P ＜ 0.05).Conclusion Imbalance between Notch pathway and lymphocytes and cytokines in children with JIA may play an important role in the pathogenesis of JIA.