A Cohort Study on Deficiency of ADA2 from China.

PURPOSE: Deficiency of adenosine deaminase 2 (DADA2), an autosomal recessive autoinflammatory disorder caused by biallelic loss-of-function variants in adenosine deaminase 2 (ADA2), has not been systemically investigated in Chinese population yet. We aim to further characterize DADA2 cases in China. METHODS: A retrospective analysis of patients with DADA2 identified through whole exome sequencing (WES) at seventeen rheumatology centers across China was conducted. Clinical characteristics, laboratory findings, genotype, and treatment response were analyzed. RESULTS: Thirty patients with DADA2 were enrolled between January 2015 and December 2021. Adenosine deaminase 2 enzymatic activity was low in all tested cases to confirm pathogenicity. Median age of disease presentation was 4.3 years and the median age at diagnosis was 7.8 years. All but one patient presented during childhood and two subjects died from complications of their disease. The patients most commonly presented with systemic inflammation (92.9%), vasculitis (86.7%), and hypogammaglobinemia (73.3%) while one patient presented with bone marrow failure (BMF) with variable cytopenia. Twenty-three (76.7%) patients were treated with TNF inhibitors (TNFi), while two (6.7%) underwent hematopoietic stem cell transplantation (HSCT). They all achieved clinical remission. A total of thirty-nine ADA2 causative variants were identified, six of which were novel. CONCLUSION: To establish early diagnosis and improve clinical outcomes, genetic screening and/or testing of ADA2 enzymatic activity should be performed in patients with suspected clinical features. TNFi is considered as first line treatment for those with vascular phenotypes. HSCT may be beneficial for those with hematological disease or in those who are refractory to TNFi.

[Role of CD4+NKG2D+ T cells in the disease activity of juvenile idiopathic arthritis]. / CD4+NKG2D+ Tç»†èƒžåœ¨å¹¼å¹´ç‰¹å‘æ€§å ³èŠ‚ç‚Žç–¾ç— æ´»åŠ¨ä¸­çš„ä½œç”¨.

OBJECTIVES: To study the expression levels of CD4+NKG2D+ T cells and NKG2D soluble ligands, the soluble MHC class I chain-related molecules A and B (sMICA/sMICB) in the active stage and stable stage of juvenile idiopathic arthritis (JIA) and their role in the disease activity of JIA. METHODS: Nineteen children with systemic JIA and 20 children with articular JIA who were diagnosed in Children's Hospital of Chongqing Medical University from November 2019 to December 2021 were enrolled in this prospective study. Six healthy children were enrolled as the control group. After peripheral blood samples were collected, ELISA was used to measure the levels of sMICA and sMICB, and flow cytometry was used to measure the percentage of CD4+NKG2D+ T cells. Systemic Juvenile Arthritis Disease Activity Score-27 (sJADAS-27)/Juvenile Arthritis Disease Activity Score-27 (JADAS-27) was used to evaluate the disease activity in children with JIA. The Pearson correlation analysis and the receiver operating characteristic (ROC) curve were used to assess the role of CD4+NKG2D+ T cells, sMICA and sMICB in the disease activity of JIA. RESULTS: The active systemic JIA and active articular JIA groups had a significant increase in the percentage of CD4+NKG2D+ T cells compared with the control group and their corresponding inactive JIA group (P<0.05). The JIA groups had significantly higher levels of sMICA and sMICB than the control group (P<0.05), and the active articular JIA group had a significantly higher level of sMICB than the stable articular JIA group (P<0.05). In the children with JIA, the percentage of CD4+NKG2D+ T cells and the levels of sMICA and sMICB were positively correlated with sJADAS-27/JADAS-27 disease activity scores (P<0.05). The ROC curve analysis showed that sMICB had an area under the curve of 0.755 in evaluating the disease activity of JIA, with a specificity of 0.90 and a sensitivity of 0.64. CONCLUSIONS: The percentage of CD4+NKG2D+ T cells and the levels of sMICA and sMICB increase in children with JIA compared with healthy children and are positively correlated with the disease activity of JIA, suggesting that CD4+NKG2D+ T cells and NKG2D ligands can be used as potential biomarkers for evaluating the disease activity of JIA.

[A preliminary study on the role of V-domain Ig suppressor of T cell activation in juvenile idiopathic arthritis]. / Tç»†èƒžæ´»åŒ–å ç–«çƒè›‹ç™½æŠ‘åˆ¶Våž‹ç»“æž„åŸŸåœ¨å¹¼å¹´ç‰¹å‘æ€§å ³èŠ‚ç‚Žä¸­ä½œç”¨çš„åˆæ­¥ç ”ç©¶.

OBJECTIVES: To study the expression of V-domain Ig suppressor of T cell activation (VISTA) in peripheral blood of children with juvenile idiopathic arthritis (JIA) and its role in the pathogenesis of JIA. METHODS: In this prospective study, peripheral blood was collected from 47 children with different subtypes of JIA and 10 healthy children. Flow cytometry was used to measure the expression levels of VISTA, interferon-γ (IFN-γ), and tumor necrosis factor-α (TNF-α) on CD14+ mononuclear cells, CD4+ T lymphocytes, and CD8+ T lymphocytes. RESULTS: The children with JIA had a significantly lower expression level of VISTA than the healthy children (P<0.05). There was a significant difference in the expression of VISTA between the children with different subtypes of JIA, with the lowest expression level in those with systemic JIA (P<0.05). There was also a significant difference in the expression of VISTA between different immune cells, with a significantly higher expression level on the surface of monocytes (P<0.05). Correlation analysis showed that VISTA was negatively correlated with the expression of IFN-γ and TNF-α on CD4+ T cells (r=-0.436 and -0.382 respectively, P<0.05), CD8+ T cells (r=-0.348 and -0.487 respectively, P<0.05), and CD14+ mononuclear cells (r=-0.582 and -0.603 respectively, P<0.05). CONCLUSIONS: The insufficient expression of VISTA may be associated with the pathogenesis of JIA, and enhancing the immunomodulatory effect of VISTA might be one option for the treatment of JIA in the future.

Two paternal mosaicism of mutation in ELANE causing severe congenital neutropenia exhibit normal neutrophil morphology and ROS production.

Severe congenital neutropenia caused by ELANE gene mutation is a rare disease. To date, only four families were reported with mosaicism. Here we examined the morphology and function of granulocytes isolated from two patients and their mosaic fathers. Analysis of granulocytes isolated from the fathers revealed no genetic mutations. DNA extracted from fractionated peripheral blood mononuclear cells (PBMCs) and fingernails obtained from both fathers did harbor the mutation, suggesting mosaicism. Granulocytes isolated from the patients displayed significantly weaker ionomycin-induced intracellular reactive oxygen species (ROS) responses than those isolated from the fathers. Both patients showed increased expression of neutrophil elastase, whereas the mosaic fathers showed normal expression. Taken together, the results suggest that granulocytes from these SCN patients are immunocompromised, whereas those from the mosaic fathers are normal. These findings may provide new insight into disease diagnosis, prognosis, therapy and genetic counseling.

[Microbial community diversity and its characteristics in Magnolia Officinalis Cortex "sweating" process based on high-throughput sequencing].

Magnolia Officinalis Cortex has been used as a traditional Chinese herb for thousands of years in China. According to Chinese Pharmacopoeia,the processing of Magnolia Officinalis Cortex needs " sweating" or " Fahan",which was a special drying process and considered to be an important symbol for high quality and genuine medicinal materials. In this unique processing mode,Magnolia Officinalis Cortex's microbial community structure may be changed,but little is known about microbial diversity during the " sweating". In this study,to analyze the change and its change rules of microbial community of Magnolia Officinalis Cortex in the whole process of " sweating",and find out the microbial community that affects the quality of Magnolia Officinalis Cortex in the process of its " sweating",and provide a basis for further research on the microbial transformation of Magnolia Officinalis Cortex,MiSeq highthroughput sequencing was used to evaluate the microbial diversity of natural " sweating" of Magnolia Officinalis Cortex. In this research,334 genera fungi and 674 genera bacteria were identified. The dominant species weren' t obvious during the early stage of " sweating". Candida was the dominant fungal species( 45. 01%-71. 93%) during the medium " sweating" stage. Aspergillus is the dominant fungal species( 45. 83%-95. 51%) during the late stage of " sweating". Moreover,Enterobacter and Klebsiella were the primary bacterial genus( ≥56. 05%) during the middle and late stages of " sweating". In addition,the predominant bacteria in the process of " sweating" included Bacillus,Deinococcus,Sphingomonas,Hymenobacter and Jatrophihabitans. In conclusion,the microbial diversities and the main dominant fungi and bacteria in the process of " sweating" of Magnolia Officinalis Cortex were initially determined. It was also found that the metabolism of Aspergillus and Candida may be related to the character formation,which were sweet odor and brown inner surface after " sweating". The results provide a theoretical basis for the study of the influence of different microorganisms on the excellent traits formation of " sweating" Magnolia Officinalis Cortex.

Novel compound heterozygous mutations in ZAP70 in a Chinese patient with leaky severe combined immunodeficiency disorder.

In humans, the complete lack of tyrosine kinase ZAP70 function results in combined immunodeficiency (CID), with abnormal thymic development and defective T cell receptor (TCR) signaling of peripheral T cells, characterized by the selective absence of CD8+ T cells. So far, 15 unique ZAP70 mutations have been identified in approximately 20 patients with CID, with variable clinical presentations. Herein, we report the first case from China of novel compound heterozygous mutations in ZAP70 (c.598-599delCT, p.L200fsX28; c.847 C>T, R283H). The patient suffered from early-onset and recurrent infections, but showed normal growth and development without signs of failure to thrive, thus presenting as leaky SCID. The patient also had clinical manifestations of autoimmunity, such as eczematous skin lesion, inflammatory bowel disease (IBD), and intractable diarrhea, suggesting compromised T cell tolerogenic functions. Residual ZAP70 expression was identified. Immunological analysis revealed the selective absence of CD8+ T cells in the periphery and the presence of CD4+ T cells that failed to respond to phytohemagglutinin. Stimulation with lectin from pokeweed mitogen also failed to stimulate B cell proliferation in the patient. The frequency of Tfhs and Tregs in the patient was lower compared with the normal reference. Compared with the age-matched healthy control, the level of IL-17 was higher and the levels of IFN-γ, IL-4, and IL-21 were lower. Infants with selected CD8 deficiency and severe autoimmune disorders or exaggerated inflammation should be screened for ZAP70 deficiency.

Relationship between intracranial internal carotid artery calcification and enlarged cerebral perivascular space.

PURPOSE: The association between intracranial internal carotid artery (IICA) calcification and lacunes, white matter hyperintensity (WMH), and cerebral microbleeds (CMBs) has been well researched. However, enlarged cerebral perivascular space (PVS) has not yet been reported to correlate with intracranial internal carotid artery calcification. Therefore, the primary aim of this study was to investigate the relationship between IICA calcification and enlarged PVS. METHODS: A total of 189 patients with ischemic stroke in the middle cerebral artery territory who presented within 7 days of ictus from 2012 to 2015 were enrolled respectively. All patients were required to have undergone head computed tomography, magnetic resonance imaging, susceptibility-weighted magnetic resonance imaging, magnetic resonance angiography, or computed tomography angiography. Clinical characteristics were recorded. IICA calcification and enlarged PVS were semi-quantitatively evaluated, and the presence of lacunes, WMH, and CMBs was recorded. RESULTS: Of the 189 patients, 63.5% were male. Mean age of the patients was 68.6 ± 12.2 years. There were 104 patients with IICA calcification. Age, diabetes mellitus, lacunes, and white matter hyperintensity were significantly associated with IICA calcification (P < 0.05). Multivariate logistic regression analysis showed that age, diabetes mellitus, and lacunes were independent predictors of IICA calcification (P < 0.05). A lower risk of IICA calcification was found in patients with a higher enlarged PVS score (P = 0.004). CONCLUSION: Higher enlarged PVS scores were associated with a lesser degree of IICA calcification. There appears to be a relationship between reduced risk of IICA calcification and enlarged PVS.

The clinical features of autoimmunity in 53 patients with Wiskott-Aldrich syndrome in China: a single-center study.

UNLABELLED: Autoimmune disease (AD) is common in patients with Wiskott-Aldrich syndrome (WAS) and patients with WAS who has an AD usually constitute a high-risk group with poor outcome. However, knowledge of AD in WAS is limited in China. In this study, medical records of 53 patients with WAS at Children´s Hospital of Chongqing Medical University from April 2004 to January 2014 were evaluated retrospectively and 14 patients (26%) had at least one AD. Autoimmune hemolytic anemia (AIHA) was the most common and detected in 12 patients (23%), other complications included immune thrombocytopenia (n = 1), immune neutropenia (n = 1), autoimmune arthritis (n = 1), and renal injury (n = 1). No significant differences were found in the level of serum immunoglobulins and lymphocyte subsets between the AD group and non-AD group. Although eight patients with AD received hematopoietic stem cell transplantation (HSCT), three patients died of pulmonary infection after HSCT. CONCLUSIONS: AD is frequent in Chinese patients with WAS and AIHA was the most common. AD is a poor prognosis factor for WAS and should be treated as early as possible by HSCT. WHAT IS KNOWN: • Autoimmune disease is common in patients with WAS. • Manifestations, follow-up finding, and treatment approaches of autoimmune disease in Chinese patients with WAS have received less attention in the literature. What is New: • This study is firstly intended for evaluation of the clinical and immune characteristics of autoimmune disease in a large series Chinese patients with WAS. • AD is frequent in Chinese patients with WAS and AIHA is the most common.

Clinical and molecular features of 38 children with chronic granulomatous disease in mainland china.

PURPOSE: Chronic granulomatous disease (CGD) is an inherited disorder, with phagocytes failing to produce antimicrobial superoxide due to deficient NADPH oxidase activity. Mutations in the gene encoding CYBB are responsible for the majority of the CGD cases. To date, there have been no reports on large samples of children with CGD in China. Therefore, in this study, we described the clinical and molecular features of 38 suspected CGD patients from 36 unrelated Chinese families. METHODS: Clinical diagnosis was performed using dihydrorhodamine assays detected by flow cytometry. Molecular analysis was used to identify underlying CGD-causative genes. RESULTS: The mean age of onset in our 38 patients was 3.4 months, while the mean age at diagnosis was 31.7 months. Apart from recurrent pneumonia and abscesses, tuberculosis (TB) and Bacille Calmette-Guerin (BCG) infections were notable features in our cohort. Overall, 17 cases died and patient 1 did not participate in the follow-up period . In total, we identified 29 different CYBB gene mutations in 31 patients. We found NCF1 and CYBA mutations in 3 and 2 patients, respectively. In addition, we identified 31 carriers and prenatally diagnosed 4 CGD and 4 healthy fetuses. CONCLUSIONS: The results of our study demonstrate that children with BCG infections or recurrent TB infections should have immune function screening tests performed. Moreover, newborns with family histories of primary immunodeficiency diseases should avoid of BCG vaccination. Molecular analysis is an important tool for identifying patients, carriers, and high-risk CGD fetuses.

[Dynamic accumulation regulation of curcumin, demethoxycurcumin and bisdemethoxyeurcumin in three strains of curcuma longae rhizome].

The paper is aimed to study the dynamic accumulation regulation of curcumin (Cur), demethoxycurcumin (DMC) and bisdemethoxyeurcumin (BDMC) in three strains of Curcuma longa, and provide scientific references for formalized cultivation, timely harvesting, quality control and breeding cultivation of C. longa. The accumulation regulation of the three curcumin derivatives was basically the same in rhizome of three strains. The relative contents decreased along with plant development growing, while the accumulation per hectare increased with plant development growing. The accumulation of curcuminoids per hectare could be taken as the assessment standard for the best harvest time of C. longa. A3 was the best strain in terms of Cur and BDMC content.

Successful interventional treatment of post-biopsy renal artery pseudoaneurysm in pediatric patients.

BACKGROUND: With an incidence ranging from 0.01% to 1.0%, renal artery pseudoaneurysm (RAP) is a rare complication after renal biopsy, percutaneous renal surgery, penetrating trauma, and rarely blunt renal trauma. METHODS: Percutaneous renal biopsy (PRB) of native kidneys was performed in 1,500 pediatric patients under real-time ultrasonographic guidance at our institution from July 1999 to January 2011. A retrospective review of these cases revealed that 2 patients developed a post-biopsy RAP. The diagnosis of RAP was established using color duplex ultrasonography (US), contrast-enhanced computed tomography (CT) and digital substraction angiography (DSA). RESULTS: Two patients developed RAP after 1,500 PRBs were performed (0.13% incidence). In the presented cases, immediate post-bioptic ultrasound showed no abnormalities. A high index of suspicion for RAP was prompted when complications such as unexplained gross hematuria and anemia occurred and the arterial phase of CT showed a well-circumscribed hyperdense area with a contrast enhancement similar to the adjacent arterial vessels. The diagnosis was confirmed by DSA and then the feeding artery of RAP was successfully occluded. After the procedure, the patients recovered and were discharged shortly. CONCLUSION: RAP is a rare, but potentially life-threatening complication after PRB and can be treated successfully with superselective arterial embolization.

[Compare the composition and content of Arnebiae radix and the stem residues].

OBJECTIVE: To compare the composition and content of Arnebiae Radix and the stem residues. METHODS: TLC and HPLC were used to identify Arnebia, ultraviolet-visible spectrophotometry was used to determine the content of hydroxy naphthoquinone total pigment in Arnebia, HPLC was used to determine the total content of /3-P'-dimethyl acrylamide Aka Ning and shikonin. RESULTS: The number of spots of Arnebia Radix was consistent with that of the stem residues in 10 batches of medicinal materials, the former was larger and deeper in color. Their features of fingerprint are the same,at the same retention time,the peak area of radix was larger; The average content of hydroxy naphthoquinone total pigment was 3.631% in the radix, and 1.516% in the stem. The total content of beta-beta'-dimethyl acrylamide Aka Ning and shikonin in the radix and the stem were respectively 0.89% and 0.309%. CONCLUSION: The ingredients in the radix and the stem residues are the same, but the contents of root of the total pigment content of hydroxyl-naphthoquinone, beta-beta'-dimethyl acrylamide Aka Ning and shikonin are twice higher than those of the stem residues.

Anti-nuclear matrix protein 2+ juvenile dermatomyositis with severe skin ulcer and infection: A case report and literature review.

BACKGROUND: Juvenile dermatomyositis (JDM) is an idiopathic inflammatory myopathy that occurs in childhood. It is characterized by muscle weakness and a characteristic rash. Previous literature reports have rarely described JDM with severe skin ulcers and infections. CASE SUMMARY: Herein, we describe a case of a 2-year-old female patient who suffered from JDM, whose myositis-specific autoantibodies were positive for anti-nuclear matrix protein 2 antibody, with progressively worsening skin ulcers and severe infections. The patient was treated with glucocorticoids and various immunosuppressants. Nevertheless, further progression of the disease and the combination of primary disease and severe infection in the later period were fatal. CONCLUSION: In children, anti-nuclear matrix protein 2+ JDM combined with skin ulcers often indicates severe disease. In such cases, personalized treatment for the primary disease and infection prevention and control are essential.

Encapsulation of a Desmodium intortum Protein Isolate Pickering Emulsion of ß-Carotene: Stability, Bioaccesibility and Cytotoxicity.

Owing to their excellent characteristics, Pickering emulsions have been widely used in the development and the application of new carriers for embedding and for delivering active compounds. In this study, ß-carotene was successfully encapsulated in a Pickering emulsion stabilized using Desmodium intortum protein isolate (DIPI). The results showed that the encapsulation efficiencies of ß-carotene in the control group Tween 20 emulsion (TE) and the DIPI Pickering emulsion (DIPIPE) were 46.7 ± 2.5% and 97.3 ± 0.8%, respectively. After storage for 30 days at 25 °C and 37 °C in a dark environment, approximately 79.4% and 72.1% of ß-carotene in DIPIPE were retained. Compared with TE, DIPIPE can improve the stability of ß-carotene during storage. In vitro digestion experiments showed that the bioaccessibility rate of ß-carotene in DIPIPE was less than that in TE. Cytotoxicity experiments showed that DIPI and ß-carotene micelles within a specific concentration range exerted no toxic effects on 3T3 cells. These results indicate that DIPIPE can be used as a good food-grade carrier for embedding and transporting active substances to broaden the application of the protein-based Pickering emulsion system in the development of functional foods.

[Mutation screening of the F VIII gene in 10 hemophilia A families].

OBJECTIVE: To identify the F VIII gene mutations of patients and suspected female carriers in 10 Hemophilia A (HA) families, and to guide the prenatal diagnosis. METHODS: PCR, denaturinghigh performance liquid chromatogramphy (DHPLC) and DNA sequencing technologies were applied to screen the F VIII gene of 8 HA patients and 12 suspected female carriers in the 10 families. Linkage analysis was performed by using St 14(DXS 52), intron 13 (CA)n and EX18/Bcl I of the F VIII gene in the HA families. In prenatal diagnosis, we screened the same mutation found in the patients. PCR-restriction fragment length polymorphism was applied to detect the new missense mutations of F VIII gene in 100 unrelated healthy individuals to exclude the possibility of polymorphism. RESULTS: Five missense mutations, 3 frameshift mutations, 2 nonsense mutations and 2 single nucleotide polymorphism (SNP) were identified in 10 the HA families. Among them, c.878A to G, c.1015A to G, c.6870G to T, c.1282delA, c.3072_3073insT, c.4880_4881insA and c.5000G to A were novel mutations or polymorphism. No missense mutations c.878A G, c.1015A to G and c.6870G to T, were found in the 100 healthy unrelated controls. (2) Nine suspected female carriers were confirmed at the gene level. (3) X risk chromosome could be determined to in 4 HA families by genetic linkage analysis. (4) Among the four fetuses for prenatal diagnosis, 2 were normal, 1 was carrier and the remaining 1 was a patient. CONCLUSION: Six novel mutations, i.e., c.878A to G, c.1015A to G, c.6870G to T, c.1282delA, c.3072_3073insT and c.4880_4881insA, were identified in this study. PCR, DHPLC and DNA sequencing could be used to screen the gene mutations of HA patients, to carry out carrier detection and prenatal diagnosis of HA families efficiently, by combining with restriction endonuclease analysis and genetic linkage analysis.

[Synthesis of novel beta-aminoalcohols containing nabumetone moiety with potential antidiabetic activity].

Twenty five new beta-aminoalcohols containing nabumetone moiety were prepared via the reduction of potassium borohydride with a convenient and efficient procedure, starting from beta-aminoketones that have been synthesized by our group. Their chemical structures were determined by IR, MS, 1H NMR, 13C NMR, HR-MS and antidiabetic activities were screened in vitro. Preliminary results revealed that the antidiabetic activity of most beta-aminoalcohols were better than that of the corresponding beta-aminoketones. Although most compounds showed weak antidiabetic activity, the alpha-glucosidase inhibitory activity of compounds 5hd(1) and 5id(2) reached 74.37% and 90.15%, respectively, which were superior to the positive control. The relative peroxisome proliferator-activated receptor response element (PPRE) activity of five compounds were more than 60%, among them compound 5ca possessed the highest activity (112.59%). As lead molecules of antidiabetic agents, compounds 5hd(1), 5id(2) and 5ca deserve further study.

Association between high mobility group box 1 protein and juvenile idiopathic arthritis: a prospective longitudinal study.

OBJECTIVE: To analyze the levels of high mobility group box 1 (HMGB1) protein on different courses of juvenile idiopathic arthritis (JIA). METHODS: In our prospective longitudinal study, children with JIA were included with their blood samples collected at the first visit, 1-month, 3-month, and 6-month follow-up, respectively. Samples were also collected from healthy controls and children with reactive arthritis at the first visit. Levels of HMGB1 were determined using enzyme-linked immunosorbent assays. Clinical disease characteristics and routine laboratory findings were analyzed as well. RESULTS: A total of 64 children were enrolled, of whom 31 (48.4%) were female. The median age at the first visit for participants with JIA was 9.25 years (range, 1.42-15.42) and the median duration of disease was 2.38 months (range, 1.53-49.31). Serum HMGB1 levels at the first visit were significantly elevated in children with systemic JIA compared with other groups, and so were in enthesitis-related arthritis versus healthy controls. Significant correlations were established at the first visit between HMGB1 levels and duration of disease, C-reactive protein, percentage of neutrophils, and ferritin. Data from all samples revealed that serum HMGB1 levels in JIA were significantly associated with erythrocyte sedimentation rates, C-reactive protein, percentage of neutrophils, and disease activity scores. CONCLUSIONS: Serum HMGB1 may be associated with clinical disease activity of JIA and specifically increased at the first visit in children with systemic JIA, suggesting its function as a sensitive inflammatory marker. Further large-scale studies are warranted to explore its spectrum in JIA.

Comparative Efficacy and Safety of Non-Steroidal Anti-Inflammatory Drugs in Patients With Juvenile Idiopathic Arthritis: A Systematic Review and Network Meta-analysis.

OBJECTIVE: We conducted a systematic review and network meta-analysis to compare the efficacy and safety of nine non-steroidal anti-inflammatory drugs (NSAIDs) in treating patients with juvenile idiopathic arthritis (JIA). METHODS: Randomized controlled trials (RCTs) of NSAIDs for the treatment in children with JIA were searched systematically by using MEDLINE, EMBASE, and the Cochrane Library for available literature up to January 1, 2019. Bayesian network meta-analysis was used to combine direct and indirect evidence on treatment effectiveness and safety. RESULTS: Eight eligible RCTs involving 1112 patients with JIA were identified, addressing 9 interventions. The ranking probability plot based on the surface under the cumulative ranking curve (SUCRA) indicated that celecoxib (6 mg/kg twice-a-day) had the highest probability of being most effective (SUCRA = 76.4%) among four NSAIDs (celecoxib, rofecoxib, meloxicam, and naproxen). Also, rofecoxib (0.3 mg/kg once-a-day) and piroxicam demonstrated a higher probability of safety in treating children with JIA (SUCRA = 33.0% and 35.5%, respectively), compared with other interventions. CONCLUSIONS: The quality of available evidence limits the formation of powerful conclusions regarding the comparative efficacy or safety of NSAIDs used to treat JIA.

Application for proteomics analysis technology in studying animal-derived traditional Chinese medicine: A review.

Different therapeutically active ingredients, from plants, animals, and mineral sources, are prescribed as traditional Chinese medicines (TCM). TCMs, from animal sources, are rich in proteins and peptides. Different advanced proteomics technologies, such as two-dimensional gel electrophoresis (2-DE), multi-dimensional liquid chromatography (MDLC), matrix-assisted laser desorption/ionization-time of flight mass spectrometry (MALDI-TOF-MS), and isobaric tags for relative and absolute quantitation (iTRAQ), have been applied to analyze TCMs, from animal sources. This paper reviews the common proteomic techniques for analyzing animal - derived TCMs. Various scientific studies have reported the application of proteomics for locating drug targets, identifying active components, and elucidating the mechanisms of action of animal - derived TCMs. However, these researches are still at the preliminary stage. This review has also discussed the existing challenges and future directions in this field of research.