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Systemic juvenile idiopathic arthritis(sJIA) is one of the most serious critical illnesses in childhood, characterized by high fever, recurrent rash, and arthritis, etc.Children with sJIA associated-lung disease(sJIA-LD) are more severely ill and have a worse prognosis, the correlation between the mechanism and age, disease activity, anti-rheumatic drug therapy, applications of biologics, infection and other factors is worth exploring.This article reviews the research progress on the mechanism, risk factors, treatment methods and prognosis of sJIA-LD, so as to provide a theoretical basis for improving the diagnosis and treatment of sJIA and improving the prognosis.
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The allergen nasal provocation testing（NPT）, in which allergens are applied directly to the nasal mucosa under standard and controlled conditions to provoke the main symptoms of allergic rhinitis（AR）, reproduces the response of the upper respiratory tract to natural exposure to allergens under controlled conditions and is the only test currently available to confirm nasal reactivity to allergens. It is invaluable in studying the mechanisms of AR and in assessing the response to novel anti-allergic treatments. The test may play an increasingly important role in clinical practice, especially in the identification of local AR, the diagnosis of occupational AR, the clarification of the composition of allergens, the assessment of the efficacy of AR treatment and the selection of candidates undergoing allergen immunotherapy. This article reviewed the application of NPT in the diagnosis of allergic and non-allergic rhinitis, and also introduces the indications, contraindications, advantages and limitations of NPT in evaluating nasal response.
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Humans , Allergens , Rhinitis/diagnosis , Nasal Provocation Tests , Rhinitis, Allergic/diagnosis , Nasal MucosaABSTRACT
Juvenile idiopathic arthritis(JIA) is one of the most common chronic connective tissue diseases characterized by unknown etiologic arthritis with the onset before the age of 16 years and disease course for more than 6 weeks.JIA may be accompanied by impairment of multiple organ function.Recent studies have shown the important role of the Janus kinase (JAK)-signal transducer and activator of transcription (STAT) pathway in the pathogenesis of JIA.Tofacitinib is an oral Janus kinase(JAK) inhibitor approved by Food and Drug Administration (FDA) in 2012 for the effective treatment of rheumatoid arthritis.However, there is little clinical evidence for the use of Tofacitinib in pediatrics.This review aims to clarify the mechanisms, efficacy and safety of Tofacitinib on the treatment of JIA.
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There is no specific medicine for systemic lupus erythematosus (SLE). Compared with adult-onset SLE, childhood-onset SLE is characterized by severe condition, rapid changes, and poor prognosis.Glucocorticoids are the first-line drugs for SLE.However, some are still difficult to be controlled using the conventional drugs like glucocorticoids and immunosuppressive agents.Belimumab is the only biological agent approved for the use in both adults and childhood-onset SLE, although its application in children lacks clinical experiences.This study aims to review the application of Belimumab in childhood-onset SLE.
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Characterized by chronic arthritis, juvenile idiopathic arthritis (JIA) refers to a group of rheumatic diseases in children with unknown causes and strong clinical heterogeneity.Most patients have good prognosis, while some may be involved with important extra-articular organs and tissues, including cardiac system damage, pulmonary or pleural lesions, renal amyloid degeneration, uveitis, and so on.Delay in diagnosis and treatment may lead to short-term and/or long-term involvement in vital systems, thus resulting in the aggravation of the disease or poor prognosis, which may decrease their life quality and even threaten their life.Understanding the extra-articular manifestations of JIA is very important to manage disease.
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Advances in high-throughput sequencing (HTS) have fostered rapid developments in the field of microbiome research, and massive microbiome datasets are now being generated. However, the diversity of software tools and the complexity of analysis pipelines make it difficult to access this field. Here, we systematically summarize the advantages and limitations of microbiome methods. Then, we recommend specific pipelines for amplicon and metagenomic analyses, and describe commonly-used software and databases, to help researchers select the appropriate tools. Furthermore, we introduce statistical and visualization methods suitable for microbiome analysis, including alpha- and beta-diversity, taxonomic composition, difference comparisons, correlation, networks, machine learning, evolution, source tracing, and common visualization styles to help researchers make informed choices. Finally, a step-by-step reproducible analysis guide is introduced. We hope this review will allow researchers to carry out data analysis more effectively and to quickly select the appropriate tools in order to efficiently mine the biological significance behind the data.
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The current document is based on a consensus reached by a panel of experts from the Chinese Society of Allergy and the Chinese Society of Otorhinolaryngology-Head and Neck Surgery, Rhinology Group. Chronic rhinosinusitis (CRS) affects approximately 8% of Chinese adults. The inflammatory and remodeling mechanisms of CRS in the Chinese population differ from those observed in the populations of European descent. Recently, precision medicine has been used to treat inflammation by targeting key biomarkers that are involved in the process. However, there are no CRS guidelines or a consensus available from China that can be shared with the international academia. The guidelines presented in this paper cover the epidemiology, economic burden, genetics and epigenetics, mechanisms, phenotypes and endotypes, diagnosis and differential diagnosis, management, and the current status of CRS in China. These guidelines—with a focus on China—will improve the abilities of clinical and medical staff during the treatment of CRS. Additionally, they will help international agencies in improving the verification of CRS endotypes, mapping of eosinophilic shifts, the identification of suitable biomarkers for endotyping, and predicting responses to therapies. In conclusion, these guidelines will help select therapies, such as pharmacotherapy, surgical approaches and innovative biotherapeutics, which are tailored to each of the individual CRS endotypes.
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Adult , Humans , Asian People , Biomarkers , China , Consensus , Diagnosis , Diagnosis, Differential , Drug Therapy , Eosinophils , Epidemiology , Epigenomics , Genetics , Hypersensitivity , Inflammation , International Agencies , Medical Staff , Neck , Phenotype , Precision MedicineABSTRACT
Allergic rhinitis (AR) is a global health problem that causes major illnesses and disabilities worldwide. Epidemiologic studies have demonstrated that the prevalence of AR has increased progressively over the last few decades in more developed countries and currently affects up to 40% of the population worldwide. Likewise, a rising trend of AR has also been observed over the last 2–3 decades in developing countries including China, with the prevalence of AR varying widely in these countries. A survey of self-reported AR over a 6-year period in the general Chinese adult population reported that the standardized prevalence of adult AR increased from 11.1% in 2005 to 17.6% in 2011. An increasing number of original articles and imporclinical trials on the epidemiology, pathophysiologic mechanisms, diagnosis, management and comorbidities of AR in Chinese subjects have been published in international peer-reviewed journals over the past 2 decades, and substantially added to our understanding of this disease as a global problem. Although guidelines for the diagnosis and treatment of AR in Chinese subjects have also been published, they have not been translated into English and therefore not generally accessible for reference to non-Chinese speaking international medical communities. Moreover, methods for the diagnosis and treatment of AR in China have not been standardized entirely and some patients are still treated according to regional preferences. Thus, the present guidelines have been developed by the Chinese Society of Allergy to be accessible to both national and international medical communities involved in the management of AR patients. These guidelines have been prepared in line with existing international guidelines to provide evidence-based recommendations for the diagnosis and management of AR in China.
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Adult , Humans , Asian People , China , Comorbidity , Developed Countries , Developing Countries , Diagnosis , Epidemiologic Studies , Epidemiology , Global Health , Hypersensitivity , Prevalence , Rhinitis, AllergicABSTRACT
<p><b>OBJECTIVE</b>To evaluate the efficacy and safety of humanized anti-IL-6 receptor monoclonal antibody (tocilizumab) in treatment of systemic juvenile idiopathic arthritis (sJIA).</p><p><b>METHODS</b>Thirteen sJIA patients admitted between December 2015 and November 2016 and received tocilizumab treatment were enrolled in the study. The complete blood count (CBC), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), interleukin-6 (IL-6) and ferritin levels were measured; American College of Rheumatology Pediatric(ACR Pedi)30/50/70/90 scores were assessed; and the use of glucocorticosteroid and adverse events were documented.</p><p><b>RESULTS</b>Compared with the baseline levels, the CRP and ESR at d3 were decreased (all<0.05); hemoglobin was increased and platelet was decreased at week 2 (all<0.05), ferritin decreased at week 4, white blood cell (WBC) decreased at week 8 after treatment with tocilizumab (all<0.05). The level of IL-6 was rising at d3 and week 2 and descending at week 4, but no significant difference was observed compared with the baseline level (all>0.05). All 13 patients achieved ACR Pedi 30 remission at week 4, 61.5% achieved ACR Pedi 90 remission and glucocorticosteroids were withdrawn at week 20. Twenty two adverse events occurred, and infection accounted for 54.5% (12/22); no severe adverse reactions were observed during 20-week follow-up.</p><p><b>CONCLUSIONS</b>Tocilizumab is safe and effective in treatment of sJIA, with decreasing inflammation, improving disease activity and reducing glucocorticosteroid use.</p>
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Autoinflammatory diseases (AID) in childhood is one of refractory diseases, whose pathogenesis is not completely clear. In recent years, a large number of studies have shown that NLRP3 inflammasome plays an important role in the development of AIDs in children. Inflammasome is a cytosolic multiprotein complex that can activate cysteinyl aspartate-specific protease-1 (caspase-1), to further promote the maturation and secretion of proinflammatory cytokines IL-1β and IL-18 as well as pyroptosis and regulate innate immune response. IL-1 receptor antagonist (Anakinra) and IL-1β monoclonal antibody (Canakinumab) have good therapeutic effects in children with AIDs. This article reviews the research progress of NLRP3 inflammasome in the pathogenesis of autoinflammatory diseases.
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Rheumatoid arthritis ( RA ) and systemic lupus erythematosus ( SLE ) are the most important and typical autoimmune diseases,however their exact mechanisms has not yet been clear. Autohpagy,a highly conserved process,depends on catabolism of lysosomal degradation of macromolecules or organelles within the cell itself. Autophagy exists widely in various tissues and is involved in maintaining survival and differentiation of cell or the stablility of internal environment. Recently,autophagy has gained wider attention and been one of the hot points of relative reserches. In this review we′ll sum up the development of autophagy in RA and SLE.
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<p><b>OBJECTIVE</b>To summarize and analyze the clinical features, diagnosis, surgical approaches and treatment outcomes of patients with primary nasal sinus osteoma.</p><p><b>METHODS</b>A retrospective review of 48 cases with primary nasal sinus osteoma treated from January 2007 to December 2013 was performed. All patients underwent preoperative CT scan and postoperative histopathologic examination. The surgical approaches included lateral rhinotomy in 14 cases, nasal endoscopic resection in 12 cases, coronal surgical incision craniotomy in 13 cases, combined craniofacial approach in 4 cases, and Caldwell-Luc approach in 5 cases.</p><p><b>RESULTS</b>The postoperative pathological diagnosis consisted of 3 variants, including 20 for compact type and 15 for cancellous type, and 13 for mixed type. Six cases were lost to follow-up and 42 cases were followed up for 6-60 months, 5 cases recurred. The post-operative complications included sinus mucous cyst in 4 cases, cerebrospinal fluid leak in 3 cases.</p><p><b>CONCLUSIONS</b>Nasal sinus osteoma are common. CT or MRI is helpful to evaluate the osteoma size, location and possible sources, and to make operation scheme. Surgery is the first choice for sinus osteoma. Lateral rhinotomy and nasal endoscopic resection can be applied to most sinus osteoma. The prognosis of sinus osteoma is good, with fewer recurrence.</p>
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Humans , Craniotomy , Endoscopy , Magnetic Resonance Imaging , Mucocele , Nose , Osteoma , Diagnosis , Pathology , Paranasal Sinus Neoplasms , Diagnosis , Pathology , Paranasal Sinuses , Pathology , Retrospective Studies , Tomography, X-Ray Computed , Treatment OutcomeABSTRACT
OBJECTIVE@#To detect the levels of Epstein-Barr virus (EBV) latent membrane protein 1 Antibodies (LMP1-Ab) in nasopharyngeal carcinoma (NPC)sera and discuss the clinical significance of this test in diagnosis, prognosis, and immune-targeted therapy of NPC.@*METHOD@#Enzyme-linked immunosorbent assay (ELISA) and Western blot method were used to detect the LMP1-Ab levels in 61 NPC sera, 30 nasopharyngitis sera, and 55 normal sera. The relationship between the LMP1-Ab level and clinical and pathological features of NPC was analyzed.@*RESULT@#ELISA test showed that LMP1 antibodies level was significantly higher in nasopharyngeal carcinoma group than those in nasopharyngitis group and in healthy group and there were statistical significances (all P0.05). Western blot test revealed that the expression of LMP1 antibodies was higher in NPC sera than in nasopharyngitis sera and in normal sera.@*CONCLUSION@#LMP1-Ab level was higher in nasopharyngeal carcinoma group than in nasopharyngitis group and in normal group. Therefore, LMP1 may be considered as a tumor correlated antigen to help the diagnosis and immune-targeted therapy of NPC.
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Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Antibodies, Viral , Blood , Carcinoma , Case-Control Studies , Enzyme-Linked Immunosorbent Assay , Herpesvirus 4, Human , Allergy and Immunology , Nasopharyngeal Carcinoma , Nasopharyngeal Neoplasms , Blood , Pathology , Viral Matrix Proteins , Allergy and ImmunologyABSTRACT
Objective To investigate the clinical features and bronchoalveolar lavage (BAL)therapy of postinfectious bronchiolitis obliterans (BO) in children. Method Ten children, who had post-infectious BO from February 2009 to February 2010, received BAL therapy, and were retrospectively analyzed. The data included pathology,chnical feature,chest HRCT scan, BALF cellular, levels of blood T cell subtypes and outcome of BAL therapy. Results Adenoviruses or mycoplasma pneumoniae were the most common etiologic agents (4/10, respectively). All patients presented persistent or recurrent dyspneic respirations and wheezing since the initial lung infection. The findings of HRCT included mosaic pattern of perfusion (6/10), accompanied by gas retention,bronchiectasis, atelectasis and bronchial wall thickening. The percentage of neutrophils in BALF was significantly increased in all cases (10/10). There were predominance of CD8+ T cell subtype (9/10) and lower ratio of CD4 +/CD8+ ( 10/10)in blood. Reduced symptoms and shortened hospital stay of BO in 9 of all 10 cases were observed after BAL therapy. Conclusions Severe adenovirus or mycoplasma pneunoniae bronchiolitis and/or pneumonia has higher risk for developing BO in children. Increased percentage of neutrophils in BALF and predominance of CD8 +T cell subtype may play an important role in the mechanism of BO. BAL therepy can reduce the respiratory symptoms of BO in children.
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AIM: To evaluate the contribution of inducible nitric oxide synthase (iNOS) and nitrotyrosine to acute lung injury (ALI) in rats with meconium aspiration. METHODS: 16 health male Sprage-Dawley rats were randomized to control group and meconium group, followed by intratracheally administration of 1 mL/kg saline or 1 mL/kg 20% human newborn meconium suspension. The animals were killed after 24 h of treatment. The measurements included bronchoalveolar lavage fluid (BALF) cell count, pulmonary myoloperoxidase (MPO) activity and nitric oxide (NO) level. Western bloting was used to determine the expression of pulmonary nitrotyrosine-a specific “footprint” of peroxynitrite and iNOS. RESULTS: Compared to control group, the rats in the meconium group had increased BALF cell counts (4.04?1.01)?10~9cells/L vs (0.53?0.19)?10~9cells/L:, pulmonary MPO activity (1.49?0.22)U/g wet lung tissue vs (0.62?0.16) U/g wet lung tissue:, NO level (12.77?5.00) mmol/g protein vs (4.89?1.32) mmol/g protein:, increased expression of nitrotyrosine and iNOS (0.46?0.19 and 1.49?0.60 vs 0.15?0.04 and 0.09?0.04, respectively), all P
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AIM: To evaluate the role and mechanisms of recombinant human superoxide dismutase (rhSOD) in meconium-induced acute lung injury (ALI) by evaluating pulmonary MIP-1? and NF-?B expression. METHODS: 24 health male Sprage-Dawley rats were randomized to 3 groups (8, each group), followed by intratracheal (IT) administration with (1) saline at (1 mL/kg) (control group); (2) 20% human newborn meconium suspension at 1 mL/kg, followed by saline at 1 mL/kg (Mec/saline group); (3) 20% human newborn meconium suspension at 1mL/kg, followed by rhSOD at 20 mg/kg (Mec/rhSOD group). The animal was killed 24 h after treatment. The measurements included the bronchoalveolar lavage (BAL) cell count, RT-PCR analysis of pulmonary MIP-1? mRNA expression, Western blotting analysis of pulmonary NF-?B expression. RESULTS: Meconium-induced ALI was characterized by increased BAL cell count, increased expressions of pulmonary MIP-1? mRNA and NF-?B protein [(4.68?1.40)?10~9 cells/L vs (0.53?0.19)?10~9 cells/L, 3.60?0.75 vs 1.56?0.33, 0.72?0.31 vs 0.23?0.12, respectively in control rats, all P
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AIM:Methylglyoxal (MG) is considered as a very active free radical,which is an important contributor to diabetic complications such as cataracts. The purpose of this study was to investigate the serum MG levels and protective effects of soybean isoflavones on streptozotocin induced diabetes. METHODS:Diabetes was induced in male Sprague -Dawley rats by intraperitoneal injection of 100 mg/kg streptozotocin (STZ). Diabetic rats were then randomly di-vided into 3 groups and received a special diet supplemented with casein (diabetes),low -isoflavone soy protein (diabetes + LIS),or high-isoflavone soy protein (diabetes + HIS) for 8 weeks,respectively. RESULTS:Compared to the diabetes or diabetes + LIS groups,diabetes + HIS diet significantly increased serum insulin levels,and reduced serum glucose,HbA1c and methylglyoxal levels (P