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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.
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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). Conclusion Compared with midazolam, dexmedetomidine had better efficacy in the treatment of severe multiple trauma in children and reduce the level of inflammation.
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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.
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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.
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OBJECTIVE:To observe the effect of different polarity of components of Rhizoma Polygonati Odovati on the spleen and thymus gland in D-galactose-induced aging-model mice so as to establish the anti-aging mechanism and anti-aging active components of Rhizoma Polygonati Odovati.METHODS:Different polarity of extracts of Rhizoma Polygonati Odovati were extracted respectively with benzin petroleum,acetic ether,dehydrated alcohol,80% ethanol,and water as extract solvents.The model mice were intragastrically administered with different polarity of components of Rhizoma Polygonati Odovati for 56 consecutive days,and the effects of each extractive on the indexes and pathological change of immune organ D-galactose-induced aging-model mice were monitored.RESULTS:Compared with each other extractive,aqueous extract of Rhizoma Polygonati Odovati can prevent the atrophy of thymus and spleen,and ameliorate the patho-constitution of spleen and thymus gland.CONCLUSION:Aqueous extract of Rhizoma Polygonati Odovati maybe the anti-aging active components in Rhizoma Polygonati Odovati,and its mechanism maybe related to its function to prevent the atrophy of immune organs and improve the immune function.
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Objective To explore the anti-aging mechanisms of Rhizoma Polygonati Odorati(RPO)and its anti-aging active components.Methods After drenching extracts of RPO for 56 days,the behavioral indexes of memory,learning and tolerance,changes of immune organs of spleen and thymus gland,testis index,plasma superoxide dismutase(SOD) activity,hepatic malondialdehyde(MDA)content were observed in D-galactose-induced suhacute senile mice model. Results Aqueous extract shows a trend of improving the memory and learning abilities,and can increase the tolerance of aging-model mice,prevent the atrophy of thymus gland and testis,enhance the activity of SOD and reduce the content of MDA.Conclusion Aqueous extract of RPO may be the anti-aging active components in RPO,and its mechanism may be concerned with the increase of immune function,prevention of the atrophy of genitical glands,counteraction of oxidation,clearance of free radicals and prevention of senile dementia.