Objective:Four cases with NLRP3-related autoinflammatory diseases were reported to summarize the clinical characteristics, genotype, and treatment responses of the disease, and to improve clinical pediatricians' understanding of the disease.Methods:A retrospective analysis was performed on 4 cases with NLRP3-related autoinflammatory diseases diagnosed in Children's Hospital of Anhui Province in 2016—2021, and the clinical features and treatment progress of NLRP3-related autoinflammatory diseases were retrospectively analyzed based on the clinical features, gene reports, and literature review.Results:① All 4 cases were male. Cases 1, 2, and 3 had the disease onset after birth, and case 4 had the disease onset 6 months after birth. All showed periodic fever, repeated urticaria-like rash, protruding forehead, and saddle nose. White blood cells count, erythrocyte sedimentation rate, and C-reactive protein were increased during the attack period, and those in the interval period were normal, and antibiotic treatment was ineffective. ② The genetic test of all these 4 children showed NLRP3 mutation. Children 1, 2, and 3 were heterozygous mutations, and their parents were wild-type. The mutation was located at chromosome Chr1: 247587658, exon c913 (exon3). G>A, the 305th aspartic acid (Asp) of the protein was changed to asparagine (Asn) in child 1. The mutation was located at the chromosomal Chr1: 247588072, the nucleic acid was changed to c1327(exon3)T>C, and the amino acid was changed to p.Y443H in cases 2 and 3. Somatic heterozygous mutation was found in case 4, and the child's parents were wild-type. In this case, the mutation was located at chromosomal Chr1: 247587658, exon3 G>A, and the 305th Asp of the protein was changed to Asn. ③Children in cases 1, 2, and 3 were treated with glucocorticoids and non-steroidal anti-inflammatory drugs at the initial stage, but the effects were limited. After receiving IL-1 antagonist treatment fever, skin rash, joint swelling and pain disappeared, and the inflammatory indexes were returned to normal. The child 4 received non-steroidal anti-inflammatory drugs and methotrexate, but he failed to respond to the treatment. Treatment with tocilizumab was not effective, however, fever, skin rash, or joint pain disappeared after treated with Khanna.Conclusion:①NLRP3-related autoinflammatory diseases can cause periodic fever, urticaria, joint involvement, and severe involvement of the central nervous system and organ amyloidosis. Which are early misdiagnosis is prone to systemic juvenile idiopathic arthritis. ②The disease was an inflammatory disease mediated by interleukin-1. At present, non-steroidal anti-inflammatory drug, glucocorticoid and chronic anti-rheumatic drugs have limited effects. IL-1 antagonists are effective and safe in the treatment of the disease.

Objective? To investigate the effects of Ramsay sedation score on the nursing intervention for children undergoing continuous renal replacement therapy(CRRT). Methods? By retrospective non-random sampling, a total of 30 children treated with CRRT in Anhui Children＇s Hospital from January 2016 to May 2017 were assigned into the control group and nursed according to the standard operating procedures of CRRT for children. Another 30 children treated with CRRT from June 2017 to September 2018 were put into the observation group and the Ramsay sedation score was applied to adjust the sedation administration plan according to the results and doctors＇ prescriptions, and nursing intervention was given at the early stage of agitation. Machine alarm, complications and adverse events were compared between the two groups. Results? After nursing intervention with Ramsay sedation score, the incidence of complications including machine alarm (1 time), failed bloodletting (1 time), bleeding at catheter puncture point (4 cases) and unplanned accidental termination of treatment (1 time) in the observation group were lower than those in the control group with statistical significance (P＜0.05). Conclusions? Ramsay sedation score is simple and practical to apply and it is convenient for pediatric blood purification specialized nurses to evaluate its function. It can effectively reduce incidences of CRRT machine alarm, complications and adverse events.

Objective To compare the sedation and anti-inflammatory effects of dexmedetomidine and midazolam on critical ill children with multiple trauma. Methods A prospective randomized controlled trial was conducted. Sixty-five critical ill children with multiple trauma admitted to pediatric intensive care unit (PICU) of Anhui Province Children's Hospital from January 2014 to September 2016 were enrolled, who were randomly divided into dexmedetomidine group (33 cases) and midazolam group (32 cases). Children of both groups received sufentanil for analgesia. Children in dexmedetomidine group firstly received 1.0 μg/kg intravenous bolus of dexmedetomidine for 10 minutes, then continuous infusion of 0.2-0.7 μg·kg-1·h-1, while in midazolam group children received 1-5 μg·kg-1·min-1 of midazolam in continuous infusion. The goal of sedation was to maintain a Richmond agitation-sedation scale (RASS) score of -1 to 0. The level of serum interleukin (IL-6, IL-8, IL-10, IL-1β), tumor necrosis factor-α (TNF-α) and C-reactive protein (CRP) were detected by enzyme linked immunosorbent assay (ELISA) at 24, 48, 72 hours after treatment, and the duration of mechanical ventilation, ratio of continuous renal replacement therapy (CRRT), length of stay in the PICU, ratio of sepsis and multiple organ failure (MOF) and mortality were also recorded. Results Compared with midazolam, dexmedetomidine decreased the level of pro-inflammatory cytokines and increased the level of anti-inflammatory cytokines. At 24 hours after treatment, the levels of serum IL-1β, TNF-α significantly decreased and IL-10 significantly increased [IL-1β (ng/L):6.48±2.89 vs. 8.07±3.14, TNF-α (μg/L): 11.25±5.21 vs. 15.44±5.97, IL-10 (ng/L): 12.10±5.35 vs. 9.58±4.71, all P < 0.05]. At 48 hours after treatment, the levels of serum IL-6, IL-8, IL-1β, TNF-α and CRP significantly decreased and IL-10 significantly increased [IL-6 (ng/L): 209.67±80.49 vs. 336.31±123.94, IL-8 (ng/L):229.09±80.81 vs. 298.28±90.25, IL-1β (ng/L): 7.31±3.02 vs. 8.74±3.17, TNF-α (μg/L): 12.52±4.79 vs. 16.58±5.98, CRP (g/L): 47.82±24.92 vs. 72.35±31.71, IL-10 (ng/L): 12.90±5.42 vs. 10.01±4.79, all P < 0.05]. At 72 hours after treatment, the levels of serum IL-8 and CRP significantly decreased [IL-8 (ng/L): 234.64±96.24 vs. 290.28±103.97, CRP (g/L): 53.24±29.12 vs. 86.58±38.30, both P < 0.05]. Compared with midazolam, dexmedetomidine could significantly reduce the duration of mechanical ventilation (days: 4.7±1.3 vs. 6.6±2.1), length of PICU stay (days: 9.5±2.7 vs. 12.3±3.9, both P < 0.05), and the ratio of sepsis (33.3% vs. 53.1%, P < 0.05). But there were no significant differences in ratio of CRRT (18.2% vs. 18.8%), MOF (9.1% vs. 18.8%) and mortality (6.1% vs. 12.5%) between two groups (all P > 0.05). Conclusion Compared with midazolam, dexmedetomidine had better efficacy in the treatment of severe multiple trauma in children and reduce the level of inflammation.

Objective To compare the myocardial protective effects of post-treatment with sevoflurane and isoflurane on myocardial ischemia-reperfusion injury(MIRI) in adult rats.Methods Twenty-four adult male SD rats were divided into four groups (n =6) by using the random number table,control group (C),isehemia-reperfusion group (R),sevoflurane post-treatment (S) and isoflurane post-treatment group(I).The Langendorff isolated heart perfusion model was established.The heart rate(HR),left ventricular end-diastolic pressure(LLVEDP),left ventricular developed pressure(LVDP),maximum rate of rise of left ventricular pressure(LV+-dp/dtmax),and maximum rate of decrease of left ventricular pressure(LV-dp/dtmax) were recorded at the end of equilibrium perfusion,and at 30,90 min of reperfusion,respectively.At the end of infusion,1 mm3.of apical myocardial tissue was removed for observing mitochondrial structure under electron microscopy and scoring.The myocardial infarct size(MIS) in the remaining heart tissue was measured by TTC staining.Results Compared with the R group,the S and I groups showed improved cardiac function indicators,decreased MIS,and reduced mitochondrial damage after reperfusion(P＜0.05).Compared with the S group,the I group showed worse heart function,increased MIS,and more severe mitochondrial damage after reperfusion(P＜0.05).Conclusion Post-treatment with sevoflurane and isoflurane has a protective effect on MIRI in adult rats.Post-treatment with sevoflurane has a better cardioprotective effect than that with isoflurane.

Objective To investigate the effect of sevoflurane postconditioning on mitochondrial connexin 43 (Cx43) during myocardial ischemia-reperfusion (I/R) in isolated rat hearts and the role of mitochondrial ATP-sensitive potassium (mito-KATP) channels in it.Methods Forty-five adult male SpragueDawley rats,weighing 200-250 g,were used in the study.Their hearts were excised and retrogradely perfused with K-H solution in a Langendorff apparatus.The 45 isolated hearts were assigned into 5 groups (n =9 each) using a random number table:control group (group C),I/R group,sevoflurane postconditioning group (group Sev),and sevoflurane postconditioning plus 5-hydroxydecanoate (5-HD,a specific mitoKATp channel blocker) group (group Sev+5-HD) and I/R plus 5-HD group (group I/R+5-HD).The hearts were subjected to 20 main of global ischemia followed by 90 min of reperfusion to establish the model of myocardial I/R injury.From the beginning of reperfusion,the hearts were perfused with K-H solution saturated with 3％ sevoflurane for 15 min in group Sev,with K-H solution saturated with 3％ sevoflurane and containing 100 μ mol/L 5-HD in group Sev+5-HD,and with K-H solution containing 100 μ mol/L 5-HD in group 5-HD.The heart rate (HR),left ventricular end-diastolic pressure (LVEDP),left ventricular developed pressure (LVDP) and the maximum rate of increase and decrease of ventricular pressure (±dp/dtmax) were recorded at the end of equilibration (T1) and 30 and 60 min of reperfusion (T2,3).At 90 min of reperfusion,the myocardial infarct size was measured by TTC staining,and the expression of total Cx43 (tCx43)and phosphorylated Cx43 (p-Cx43) in mitochondria was determined by Western blot analysis.The percentage of myocardial infarct size and p-Cx43/tCx43 ratio were calculated.Results Compared with group C,the HR,LVDP and ±dp/dtmax were significantly decreased,and the LVEDP was increased at T2,3,and the percentage of myocardial infarct size was increased in the other 4 groups,the expression of mitochondrial tCx43 and p-Cx43 was significantly down-regulated in I/R,Sev+5-HD and 5-HD groups (P＜0.05),and no significant change was found in the expression of mitochondrial tCx43 and p-Cx43 in group Sev (P＞0.05).Compared with group I/R,the HR,LVDP and ±dp/dtmax were significantly increased,and the LVEDP was decreased at T2,3,the percentage of myocardial infarct size was decreased,and the expression of mitochondrial tCx43 and p-Cx43 was up-regulated in group Sev (P＜0.05),and no significant change was found in the parameters mentioned above in Sev+5-HD and 5-HD groups (P＞0.05).Compared with group Sev,the HR,LVDP and ±dp/dt were significantly decreased,and the LVEDP was increased at T2,3,the percentage of myocardial infarct size was increased,and the expression of mitochondrial tCx43 and p-Cx43 was down-regulated in group Sev+5-HD (P＜0.05).There was no significant change in the pCx43/tCx43 ratio between the five groups (P＞0.05).Conclusion The mechanism by which sevoflurane postconditioning attenuates myocardial I/R injury may be related to induction of mito-KATP channel opening and up-regulation of the expression of mitochondrial Cx43 in cardiomyocytes of rats.

Interleukin(IL)-35 is a new member of the interleukin-12 superfamily. Since its first report in 2007, IL-35 rapidly became a research highlight in the ifeld of immunology. Like other IL-12 superfamily members, IL-35 was a heterodimer which was composed of anαchain P35 and aβchain Epstein-Barr virus induced gene 3 (EBI3). Recent research work revealed two distinct roles of IL-35. Firstly, IL-35 is highly expressed in some kinds of inlfammatory diseases and autoim-mune diseases and plays import roles in the pathogenesis. Secondly, IL-35 is positively expressed in some cancers and plays some roles in the process of tumor progression. Here we demonstrate the structure and the signalling of IL-35. We reviewed the the roles of IL-35 in promoting tumor progression.

Objective To investigate the correlations between the clinical manifestations based on pathologic grades and renal pathological features of Henoch - Schonlein purpura nephritis(HSPN)in children. Methods The clinical data of 77 patients with HSPN in the Department of Nephrology,Anhui Provincial Children's Hospital from Ja-nuary 2004 to March 2014 were retrospectively analyzed. The relationship between clinical manifestation and pathologi-cal features was analyzed. Results Among the 77 patients,21 cases(27. 3% )had both abdominal symptoms,and ar-thritis was reported in 15 cases(19. 5% ),28 cases(36. 4% )had abdominal symptoms and arthritis,and 13 cases (16. 9% )had no such symptoms. Hematuria and proteinuria were the most common clinical types[48. 1%(37 / 77 ca-ses)],followed by simple hematuria or proteinuria[27. 3%(21 / 77 cases)],nephrotic syndrome[23. 4%(18 / 77 ca-ses)],and chronic nephritis[1. 3%(1 / 77 cases)]. The major of pathological changes in HSPN were grade Ⅱ[46. 8%(36 / 77 cases)]and grade Ⅲ[45. 5%(35 / 77 cases)],the minority of them were grade Ⅰ[6. 5%(5 / 77 cases)]and grade Ⅳ[1. 3%(1 / 77 cases)]. The severity of urine protein was positively associated with pathologic classification (r s = 0. 472,P = 0. 000). According to the glomerular deposition of immune complex,there were 6 types. The percen-tage of deposition of IgA + IgM was 62. 3%(48 / 77 cases),IgA + IgG + IgM was 19. 5%(15 / 77 cases),IgA 14. 3%(11 / 77 cases),that of IgA + IgG 1. 3%(1 / 77 cases),and the IgM 1. 3%(1 / 77 cases),no Ig 1. 3%(1 / 77 cases). In these cases,76. 6%(59 / 77 cases)had complements C3 deposition;pathologic stage characterized by Ⅲ level and a-bove were common[54. 2%(32 / 59 cases)],Ⅱ level 42. 2%(25 / 29 cases),Ⅰ level 3. 4%(2 / 59 cases). Among the different types of immune complex depositions,there was no statistically significant difference in pathological types of distribution,while the clinical type and complements C3 deposition were significantly associated with pathologic classifi-cation(rs = 0. 361,P = 0. 001). Sixty - two cases were rated as level 1(80. 5% ),and 15 cases was level 2(19. 5% );in different clinical group,rating in glomeruli was statistically different(χ2 = 17. 2,P = 0. 004). Renal tubular interstitial rating of all the patients were level 1(100% ). Conclusions The severity of urine protein,complements C3 deposition is associated with pathologic classification. Pathologic classification can basically reflect the renal damage in HSPN.

Objective To explore the effect of heating temperature and heating time on the dissolution characteristics of phenolic acids in water extract of Danshen Decoction. Methods The contents of tanshinol, protocatechuic aldehyde and salvianolic acid B in water extract of Danshen Decoction were determined by reverse-phase high performance liquid chromatography (RP-HPLC) . The changes of the contents were monitored by classic constant temperature acceleration method. Results Of the three components, the content of salvianolic acid was decreased with the increase of heating temperature and the prolongation of heating time, and the degradation characteristics of the salvianolic acid B met the first-order kinetics equation. Conclusion The water-soluble components have less stability in the water extracts of Danshen Decoction. Lignum Santali Albi and Fructus Amomi accelerate the degradation speed of Radix Salviae Miltiorrhizae, suggesting that we should pay attention to the influence of heating temperature and heating time on the content of salvianolic acid B in the extraction and concentration process.

Objective: To study the efficacy and safety of the intrapleural injection of nedaplatin combined with recombinant hu-man endostatin (Endostar) in the treatment of malignant pleural effusion in non-small cell lung cancer (NSCLC) patients. Methods:Seventy patients diagnosed with NSCLC with malignant pleural effusion were treated by intrapleural injection. The subjects were divid-ed into two groups, as follows: 35 cases were injected with nedaplatin and Endostar (the treatment group) and 35 cases were injected with nedaplatin only (the control group). One month after the treatment, the clinical efficacy and toxicity were evaluated. The clinical ef-ficacy, quality of life, and toxic reaction were compared between the two groups. Results: The short-term clinical efficacy rate of the treatment group was 74.28%, whereas that of the control group was 48.57%, and the difference between the two groups was significant (P<0.05). Adverse effects such as nausea vomiting and leukopenia, occurred in both groups, but no significant differences were found between the two groups (P>0.05). Conclusion: The efficacy of the intrapleural injection of recombinant human endostatin combined with nedaplatin is superior to that of cisplatin alone in the treatment of malignant pleural effusion, without increasing chemotherapeutic toxicity.

Objective:To determine the relationship among tumor malignancy, invasion, and plasma D-dimer (DD) levels via a retrospective analysis of the correlation between preoperative plasma DD levels and postoperative pathology parameters in esophageal cancer patients. Methods:A total of 101 esophageal cancer patients without coagulation among preoperative plasma DD levels and postoperative pathology parameters in esophageal cancer patients who underwent radical operation between January 2009 to April 2010 were enrolled. The preoperative plasma DD levels, postoperative pathology T, N Grade, number of metastatic lymph nodes, tumor posi-tion, tumor length, and TNM stage were recorded. A statistic analysis of the above parameters was performed to reveal the correlation between plasma DD levels and tumor pathology features. Results:Preoperative plasma DD level has no correlation with Grade and tu-mor length, but shows positive correlation with pathology T, N status, and TNM stage, and has strong positive correlation with numbers of metastatic nodes. In addition, lower-third thoracic esophageal cancer patients have higher DD level than middle-third thoracic cases. Conclusion:Plasma DD level has a positive correlation with T stage, N stage, TNM stage, and number of positive lymph nodes. It also has important clinical significance.