Clinical characteristics of 86 children with Beh?et′s disease / 中华风湿病学杂志

Objective:To analyze the clinical characteristics of pediatric patients with Beh?et′s disease.Methods:The clinical characteristics of 86 newly diagnosed children with Beh?et′s disease admitted to the rheumatology department of Beijing Children′s Hospital from July 2015 to December 2020 were analyzed retrospectively. Statistical product and service solutions (SPSS) 26 was used for statistical analysis. The normal distribution of measurement data is expressed in Mean± SD, and the non normaldistribution of measurement data was expressed in median(minimum, maximum). The counting data was expressed in frequency (cases) and percentage. Results:There was no gender difference in the incidence of Beh?et′s disease in 86 children.The age of onset was 0.1~15.9 years, with an average of (7±4) years, and the age of diagnosis was 1.3~16.6 years, with an average of (10±4) years.The course of disease from onset to diagnosis was 0.5~168 months, with a median course of 21 months. Among 86 cases, 52 cases (60.5%) showed the most common oral ulcer at the onset, followed by 19 cases (22.1%) with fever. In terms of clinical manifestations: the most common clinical manifestation was oral ulcer in 82 cases (95.3%), followed by fever in 58 cases (67.4%), and gastrointestinal symptoms in 44 cases (51.2%). The common manifestation of digestive system involvement was abdominal pain and diarrhea. Ten cases (11.6%) had ocular symptoms, 13 cases (15.3%) had vascular involvement, and 3 cases (3.5%) had pulmonary involvement. Fourteen cases (16.2%) had family history. Fourty seven patients (54.7%) had elevated leukocyte, 65 patients (75.6%) had elevated CRP and 72 patients (83.7%) had elevated ESR.Conclusion:Beh?et′s disease in children is usually insidious in onset and infants may suffer from this disease. Oral ulcer is the most common clinical manifestation, followed by fever. For patients with fever of unknown cause, Beh?et′s disease should be noted. In terms of involvement of important organs, digestive tract involvement is more common in childhood, followed by large blood vessels and eyes.

Research progress of juvenile dermatomyositis with anti-melanoma differentiation-associated gene 5 antibody / 中华实用儿科临床杂志

Juvenile dermatomyositis (JDM) is the most common subtype of juvenile idiopathic inflammatory myopathy (JIIM), characterized by non-suppurative inflammation of skin and muscle.JDM frequently involves important organs such as lungs.JDM with anti-melanoma differentiation-associated gene (MDA) 5 antibody has unique clinical characteristics, mainly including skin mucosal ulcer, palm papule, hair loss and arthritis.Interstitial lung disease (ILD) is its most serious complication.The levels of serum ferritin, Krebs von den Lungen-6 and interleukin-18 can be used as important indicators of disease activity and prognosis.Glucocorticoids combined with immunosuppressants are the basic treatment for the disease.Immunosuppressants include calcineurin inhibitors (Cyclosporine A and Tacrolimus), Cyclophosphamide, Azathioprine, Mycophenolate Mofetil, etc.Refractory patients can also be treated with Rituximab, Janus kinase inhibitor and human immunoglobulin.Early active treatment of JDM with anti-MDA 5 antibody can alleviate the symptoms, reverse organ damage and improve the long-term prognosis.

The organ involvement and autoantibodies in children with systemic lupus erythematosus / 中华风湿病学杂志

Objective:To explore the correlation between autoantibodies and organ involvement in children with systemic lupus erythematosus (SLE).Methods:From June 2006 to October 2020, 581 children with SLE who were hospitalized in Beijing Children's Hospital for the first time and had autoantibody detection and clinical data in our hospital were selected. A correlation study was carried out on the clinical manifestations and autoantibodies. Data were analyzed with Pearson χ2 or Fisher's exact test. P<0.05 was considered statistically significant. Results:A total of 581 children with SLE were included in this study, with a male to female ratio of 1∶3.6. The average age at diagnosis was (10.6±2.8) years, and the main symptoms were rash (388, 66.8%), fever (335, 57.7%), and joint swelling and pain (170, 29.3%). The most commonly affected organ is the blood system (414, 71.3%), followed by lupus nephritis (257, 44.2%) and arthritis (170, 29.3%). In this study, the positive rate of ANA was 100%, and the positive rates of anti-dsDNA antibody and anti-Sm antibody were 59.7% and 21.2%, respectively. The children with anti-dsDNA antibody positive were more likely to have fever (64.6% vs 47.4%, χ2=16.77, P<0.001), and the kidneys (53.3% vs 30.8%, χ2=28.80, P<0.001) and blood systems (76.1% vs 64.1%, χ2=9.79, P=0.002) were more likely to be involved than anti-dsDNA antibody negative. The proportion of renal involvement (27.8% vs 47.5%, χ2=12.69, P<0.001), blood system (57.7% vs 74.0%, χ2=10.40, P=0.001), lung involvement (12.4% vs 21.1%, χ2=3.88, P=0.049) and cardiac involvement (9.3% vs 17.8%, χ2=4.11, P=0.042) in patients with anti-SSB antibody positive were lower than those in patients with anti-SSB antibody negative. Anti-histone antibody-positive patients were prone to lupus nephritis (56.9% vs 36.6%, χ2=22.62, P<0.001), arthritis (37.6% vs 24.2%, χ2=11.77, P=0.001) and lung involvement (24.3% vs 16.8%, χ2=4.87, P=0.027). Anti-Sm antibody positive patients were prone to skin manifestations such as butterfly erythema (52.8% vs 31.7%, χ2=11.38, P<0.001) and sunlight allergy (13.8% vs 7.4%, χ2=4.96, P=0.026), but the proportion of joint involvement (22.0% vs 31.2%, χ2=4.03, P=0.045) and thrombocytopenia (17.1% vs 27.3%, χ2=5.38, P=0.026) were lower than those of anti-Sm antibody negative. Arthritis (44.4% vs 24.8%, χ2=19.00, P<0.001), secondary SS (28.6% vs 5.4%, χ2=57.98, P<0.001) and parotid gland involvement (25.6% vs 2.9%, χ2=70.84, P<0.001) were more common in RF factor positive children, but the proportion of kidney involvement (30.8% vs 48.2%, χ2=12.57, P<0.001) was lower than in RF negative children. Conclusion:The clinical manifestations of childhood SLE are diverse and highly heter-ogeneous. A variety of autoantibodies are associated with organ involvement and clinical phenotypes, and anti-SSB antibodies may have protective effects in kidney and other organ damage.

Clinical Characteristics and Treatment of Blau Syndrome in Chinese Children-a National Multicenter Study / 罕见病研究

  Objective  To study the demographic and clinical characteristics, correlation of genotype and phenotype and treatment of Blau syndrome to facilitate early diagnosis and timely treatment of Blau syndrome.  Methods  Seventy-two patients with Blau syndrome from 11 centers from May 2006 to April 2022 were retrospectively analyzed, and their general information, clinical data, laboratory examination and treatment medication were collected.  Results  The distribution of patients with Blau syndrome was uniform in geographical north and south of China, and there was no obvious gender bias. The mean age of onset was (14.30±12.81) months, and the age of diagnosis was (55.18±36.22) months. 35% of patients with Blau syndrome happened before 1 year old, and all patients developed before 5 years old. 87.50% (63/72) had granulomatous arthritis, 65.28% (47/72) had rash, 36.11% (26/72) had ocular involvement, 27.78% (20/72) had fever, and 15.28% (11/72) had pulmonary involvement. Arthritic manifestations of Blau syndrome were most at risk, followed by rash, ocular involvement, and fever.The first 25 months of the disease, the risk of developing a rash was the greatest. The risk of developing arthritis was the greatest between 25 months and 84 months. The main mutations were p.R334Q and p.R334W, and patients with p.R334Q mutation had relatively high incidence of fever (35.71%[5/14] vs. 14.29%[1/7], P=0.43) and ocular involvement (42.86%[6/14]vs. 28.57%[2/7], P=0.51). There was a relatively high incidence of rash (85.71%[6/7] vs. 64.29%[9/14], P=0.59) in patients with the p.R334W mutation. Forty-five patients(62.50%)were treated with a combina-tion of glucocorticoid and methotrexate. Twenty-two patients were treated with tumor necrosis factor antagonist in addition to glucocorticoid and methotrexate.  Conclusions  The risk of different clinical manifestations of Blau syndrome from high to low was arthritis, followed by rash, ocular involvement and fever. The main treatment was glucocorticoid combined with methotrexate, to which biological agents could be added.

Recommendations of diagnosis and treatment of juvenile idiopathic arthritis in China / 中华内科杂志

The common clinical subtypes of juvenile idiopathic arthritis (JIA) include systemic onset juvenile idiopathic arthritis (SOJIA), oligoarthritis/polyarthritis juvenile idiopathic arthritis and juvenile spondyloarthritis. Juvenile idiopathic arthritis has no specific diagnostic index, and needs to be differentiated from infectious diseases and malignant diseases. The onset of SOJIA is rapid, the disease progresses rapidly, and it is easy to be complicated with macrophage activation syndrome (MAS) which is life-threatening. The experience of pediatric rheumatologists in dealing with JIA is still insufficient, and the standardized diagnosis and treatment level of this disease needs to be further improved. Based on the experience and guidelines of diagnosis and treatment in China and abroad, we formulated this diagnosis and treatment standard, aiming at standardizing the diagnosis and treatment of the subtypes of JIA and MAS, so as to reduce the incidence of disability and serious complications and improve the prognosis.

Clinical significance of group A streptococcal infection in pediatric patients with enthesitis related arthritis / 中华风湿病学杂志

Objective:To demonstrate the clinical significance of group A streptococcal infection (GAS) in patients with enthesitis related arthritis (ERA).Methods:A retrospective study was conducted on ERA (136) and PolyRF-/Oligo juvenile idiopathic arthritis (JIA) (272) patients in Beijing Children's Hospital from 2016 to 2018. Anti-streptococcal hemolysin "O" (ASO) was tested and documented in all patients. The infection rate of GAS was compared between patients with ERA and PolyRF-/Oligo JIA. Patients with ERA were divided to two groups according to the result of ASO (ASO positive and ASO negative). All the clinical data were documented and compared within the two groups. The statistical methods used mainly include t test, rank sum test, chi-square test, and Spearman correlation analysis.Results:The GAS infection rate of patients with ERA was higher than patients with PolyRF-/Oligo JIA (17.6% vs 9.5%, χ2=5.52, P=0.019). In ERA patients, clinical data were analyzed, and a statistical significant difference was observed in the presence of human leukocyte antigen (HLA)-B27 between ASO positive and ASO negative group [75.0%(18/24) vs 49.1%(55/112), χ2=5.329, P=0.021]. Statistical differences were found in Patrick's sign positive rate between the two groups [100%(24/24) vs 67.0%(75/112), χ2=10.61, P=0.001]. There was statistically significant difference between the two groups regarding the radiogr-aphic grading at the sacroiliac joint. More patients with positive ASO had grade Ⅲ damage at the sacroiliac joint compare to patients with negative ASO [68.2%(15/22) vs 28.4%(29/102), χ2=12.49, P<0.001]. The logarithmic of the ASO was slightly correlated with the radiographic grade of sacroiliac joint ( r=0.26, P=0.005). Conclusion:Patients with ERA are prone to be infected by GAS. It's probably related to HLA-B27 postivity for antigen presentation. Patients who were infected by GAS fre-quently have sacroiliac joint involvement, and tend to be more sever. This indicates that GAS may play an important role in the pathogenesis of sacroiliac joint destruction.

Curative effect and follow-up analysis of 15 children with refractory systemic lupus erythematosus treated with Belimumab / 中华实用儿科临床杂志

Objective:To analyze the outcome of 15 cases with refractory systemic lupus erythematosus (SLE) treated with Belimumab, and evaluate the safety and efficacy of the therapy.Methods:A retrospective and real-world clinical research method was adopted.Fifteen children with confirmed refractory SLE and complete follow-up data were selected from the Department of Rheumatology, Beijing Children′s Hospital from April 1, 2020 to March 31, 2022.By comparing the changes of clinical symptoms, auxiliary examination results, SLE disease activity index (SLEDAI-2000) and Physician′s Global Assessment (PGA) scores as well as adverse events in different treatment periods (before treatment, 4 weeks, 8 weeks, 12 weeks, 6 months and 12 months after treatment), the safety and effectiveness of Belimumab treatment were all recorded.The counting data was expressed in percentage, the measurement data meeting the normal distribution was expressed in Mean±SD, and the two samples of measurement data were compared by t-test, P<0.05 means significant differences. Results:The ratio of male to female was 3∶2, and the onset age was (7.93±4.99) years; The basic treatment time was 4 months to 5 years and 1 month.There were 8 cases with lupus nephritis (LN), 2 cases suffering from hypocomplementemia for more than 1 year, 2 cases with central nervous system involvements, 2 cases complicated with antiphospholipid syndrome and 1 case with early-onset SLE.Of 8 LN cases, 1 case was complicated with neuropsychiatric lupus (NPLE) and distal femoral head infarction of both knees, and 3 cases were complicated with lumbar compression fractures and hip infarction.All patients were treated with regular traditional therapy to induce remission.During the maintenance period, the disease activity maintained at light to moderate levels, and it was difficult to reduce glucocorticoid.At baseline, SLEDAI-2000 score was 4-13, and PGA score was 1-2.50.Basic treatment includes glucocorticoids combined with immunosuppressants (Cyclosporine, Mycophenolate Mofetil, Leflunomide tablets) and antimalarial drugs, and Cyclophosphamide and/or Tripterygium Wilfordii were used at the same time according to the damage of target organs.The drug safety after intravenous injection of Belizumab showed that one patient in this group had respiratory tract infection symptoms 4 weeks after treatment; Another patient had a slight increase of alanine aminotransferase 8 weeks after treatment, and recovered to normal symptomatic treatment.No drug-related adverse reactions were found in the other 13 patients.After 4 weeks of treatment, the score of SLEDAI-2000 and PGA compared with the baseline level, and the difference was statistically significant (SLEDAI-2000 P=0.002; PGA P=0.006). There was no clinical recurrence.One patient with familial chilblain like lupus erythematosus showed significant improvement in rash 2 weeks after treatment, and low fever accompanied by increased rash 8 weeks after treatment; After 16 weeks of treatment, the body temperature was normal and the rash basically subsided. Conclusions:Belimumab is clinically effective in the treatment of refractory childhood SLE, with no serious adverse events reported.However, its long-term efficacy and safety need to be further studied by multi-center and long-term research with a large sample size.

Clinical characteristics and follow-up study of 210 children with systemic lupus erythematosus / 中华实用儿科临床杂志

Objective:To evaluate the systemic involvement, outcome and other disease characteristics of children with systemic lupus erythematosus (cSLE), and to explore the prognostic factors.Methods:cSLE treated in Beijing Children′s Hospital, Capital Medical University from January 1, 2016 to December 31, 2017 were enrolled in this study.Medical records including clinical manifestations and evaluation of affected systems, autoantibodies, treatment adjustment, and follow-up were collected and analyzed retrospectively.SPSS 21.0 was used for statistical analysis and mapping.The prognostic factors were studied by the Cox proportional risk regression model.Results:A total of 210 children were included, including 37 males and 173 females, with a male to female ratio of 1.0∶4.7.The average age of onset was (121.39±30.44) months.There were 167 (79.5%) patients with skin and mucous membrane damage, 137(65.2%) patients with blood system damage, 129(61.4%) patients with digestive system damage, 123(58.6%) patients with kidney damage, 119(56.7%) patients with skeletal and musculoskeletal system damage, 71(33.8%) patients with nervous system damage, 68(32.4%) patients with heart damage, and 60(28.6%) patients with respiratory system damage.The 90.95%(191/210) of the children enrolled presented moderate or high disease activity at the first visit.The effective rate was 76.92% (150/195) after 1-month follow-up and 96.95% (159/164) after 1-year follow-up.A high level of compliment C 3 was a protective factor for disease remission.The glucocorticoid level was declined to 5 mg or less in 42 children, and the median time was 40.5 (36.0, 42.0) months.Young onset age and no renal damage were protective factors for glucocorticoid reduction. Conclusions:cSLE tends to occur in female children with multiple involved systems and severe conditions.After reasonable treatment and follow-up, the disease can be alleviated or improved in one year.A high level of complement C 3 at the beginning of disease is conducive to rapid remission of the disease, and the young age of onset and no renal damage is conducive to rapid glucocorticoid reduction.

Recommendations of diagnosis of autoinflammatory diseases in China / 中华内科杂志

Autoinflammatory diseases (AIDs) are a group of disorders characterized by dysfunction of innate immunity which caused by gene mutations leading to coded proteins changes, finally causing uncontrolled systemic inflammation. AIDs are a group of rare rheumatic and inflammatory diseases. Here, Chinese Rheumatology Association summarized manifestations of the main AIDs, and to standardize the methods for diagnosis of AIDs.

Evaluation of clinical effects of the early warning scale on systemic juvenile idiopathic arthritis complicated with macrophage activation syndrome / 中华实用儿科临床杂志

Objective:To compare the disease outcome, quality of life score [evaluated by child health assessment questionnaire - disability index(CHAQ-DI)] and medical expenses of children with systemic juvenile idiopathic (sJIA) combined with macrophage activation syndrome (MAS) diagnosed by two different criteria.And to analyze the impacts of early MAS diagnosis criteria on the prognosis of sJIA combined with MAS in children.Methods:From January 2016 to December 2020, children with high disease activity of sJIA who were diagnosed and initially treated in the Department of Rheumatology of Beijing Children′s Hospital were enrolled in this study.Clinical characteristics on admission were recorded as baselines.Patients were divided into 2 groups according to different diagnostic criteria.Children diagnosed as MAS based on the 2016 The European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation MAS diagnostic criteria were included in MAS control group(38 cases), and those diagnosed as early MAS based on the sJIA combined MAS early warning scale but did not meet the 2016 diagnostic criteria were included in MAS early warning group(38 cases). Basic information, clinical manifestations and laboratory test results were collected.According to the clinical manifestations and laboratory results in different periods of follow-up at 4 weeks, 8 weeks, 12 weeks, 6 months and 12 months after treatments, the di-sease activity, CHAQ-DI and medical expenses were compared between the two groups.Results:There were no signi-ficant differences in the disease activity, duration of sJIA and medical expenses between the two groups (all P>0.05). In terms of laboratory results, serum ferritin in MAS early warning group were significantly lower than that of MAS control group at 4 weeks after treatment[(333.97±186.66) μg/L vs.(389.66±221.76) μg/L]( t=-83.47, P<0.05). In terms of disease activity, after 12 months of treatment, the evaluation of American College of Rheumatology pediatric indexes 70 in MAS early warning group was better than that in MAS control group [34.2%(13/38 cases) vs.7.9% (3/38 cases)]( χ2=6.067, P<0.05). In terms of CHAQ-DI, at 4 weeks, 8 weeks, 12 weeks and 6 months of treatment, CHAQ-DI in MAS early warning group were better than those in MAS control group, and the difference were statistically significant ( t=-0.34, -0.27, -0.23, -0.09; all P<0.05). In terms of cumulative medical expenditure at 12 months of treatment, the MAS early warning group was lower than the MAS control group [(114.3±80.7) thousand yuan vs.(157.9±111.7) thousand yuan]( t=-3.97, P<0.05). Conclusions:Quickly judge the condition through the quantitative integral of clinical examination and test indexes, screening and treatment of MAS in early stage are helpful to improve the prognosis and reduce the medical consumption.

Clinical characteristics and follow-up of thrombosis in pediatric patients with systemic lupus erythematosus / 中华风湿病学杂志

Objective:To investigate the clinical characteristics and follow-up of thrombosis of pediatric patients with systemic lupus erythematosus (SLE).Methods:In this retrospective study, inpatients who were diagnosed in Beijing Children's Hospital with SLE complicated with arterial or venous thrombosis from January 2006 to December 2019 were collected, the clinical characteristics and outcomes were analyzed. Statistical product and Service solutions (SPSS) 25.0 was used for statistical analysis. T test or χ2 test (counting data) was used to compare the differences between groups, and Kaplan-Meier survival curve was used to analyze the time of thrombus endpoint events in lupus children. Results:A total of 1 395 newly diagnosed SLE patients were admitted. Twenty-seven cases were diagnosed with thrombosis, accounting for 1.9% of all the lupus patients. The median course from diagnosis to thrombosis was 20 days (49 days before diagnosis to 1 year after dia-gnosis). Among the 27 patients, 22(81%) cases had renal involvement. The mean SLE disease activity index (SLEDAI) score was (14±6) and (11±4) at the diagnosis of lupus and at onset of thrombosis, respectively ( t=2.547, P=0.017). 30% (8/27) of the patients had no apparent clinical manifestations of thrombosis. The patients received standard anticoagulant therapy after the diagnosis of thrombosis. During follow-up, 6 patients stopped taking medications due to the severity of the primary disease. Twenty-one patients were followed up regularly for 1-3 years. Thrombosis disappeared in 12 cases (44%), thrombolysis time ranged from 16 days to 1 year. Thrombosis were getting smaller in 9 cases (33%). And the disease was stable during follow up. Conclusion:Thrombosis is not rare in pediatric patients with systemic lupus erythematosus patients. Some patients do not have apparent clinical manifestations related to thrombus. Pediatricians should be alert to patients with renal involvement need to be more vigilant for thrombosis. Early detection and active treatment are the keys to improve the prognosis of thrombosis in pediatric SLE patients.

Clinical analysis and long-term follow-up of 612 cases with juvenile dermatomyositis / 中华风湿病学杂志

Objective:To explore the clinical characteristic and prognosis of juvenile dermatomyositis (JDM) by retrospectively study of the clinical manifestations, laboratory examinations, treatment and follow-up results. The aim of this study was to improve the diagnosis and treatment of JDM and reduce the complications and mortality.Methods:Medical charts of 612 JDM cases hospitalized to Beijing children's hospital from July 2002 to July 2018. We retrospectively analyze the onset, clinical manifestations, laboratory examinations, treatment and the follow-up, and then summarize the clinical characteristics and assess the therapeutic effect and prognosis.Results:There were 278 male and 334 female. The maleto female ratio was 1∶1.2. Themedian age at symptoms onset was 5.4(2.9-8.4) years old (range 6 months to 14 years). Rash was the most common initial presentation. The main clinical manifestations were rash (100%, 612 cases) and muscles weakness (96.1%, 588 cases). The most commonly involved organs by JDM were lung (57.5%, 352 cases), digestive tract (38.5%, 236 cases) and heart (32.5%, 199 cases). Muscle enzymes elevated in 95.5% (584 cases) of the patients and 89.5%(534 cases) of the patients had typical changes on electromyography. Muscle biopsy was performed in 134 patients and pathologicresults were compatible with JDM. For the treatment, all of the patients were treated by steroids plus therapy combined with immunosuppressive agents. Mostof the patients got good effect and outcome. Twenty-four patients died, and acute respiratory failurewas the most common cause of death. 17.9%(105 cases) of patients had complications. The complications included calcinosis in 70 patients and amyotrophy in 35 patients.Conclusion:JDM is a rare disease of children, andis characterized by muscle weaknessand rash. Severe organ involvement may cause death. Treatments include corticosteroids and immunosuppressive agents, andthe outcome is generally good.

Analyses of gene mutation, clinical phenotype, treatment and follow up of 10 cases with chronic infantile neurologic, cutaneous, articular syndrome / 中华风湿病学杂志

Objective To explore the gene mutation,clinical phenotype,treatment and prognosis of chronic infantile neurologic,cutaneous,articular (CINCA) syndrome,so as to improve the diagnosis rate,reduce the disability rate and teratogenicity rate of CINCA syndrome.Methods Ten children with CINCA syndrome admitted to our hospital were retrospectively analyzed in terms of the clinical phenotypes,auxiliary examinations,treatment and follow-up.Three ml ethylene diamine tetraacetic acid (EDTA) anticoagul-ation was taken from children and their parents with the consents.Genomic DNA was extracted by QIAamp whole blood Deoxynbonucleic acid (DNA) extraction kit (German Qiagen Company).The whole exons were detected by Agilent liquid phase capture technology (Agilent Company).Finally,Sanger sequencing was used to verify the results.Results In this study,eight mutations of NLRP3 gene were found in children with CINCA syndrome,namely 913G/A (D305N),1057G/T(V353L),1702T/A (F568I),1703T/A (F568Y),1710G/C (K570N),1789A/G (S597G),1991T/C (M664T),2269G/A (G757R).The onset age of most of the cases was less than half a month,and the initial manifestation was mainly urticaria-like rash.Short stature and special face could be seen in all 10 cases.All the patients had fever and urticarial rash in varying degrees during the course of the disease.Nine of them had obvious arthritis.Nine children had central nervous system involvement.There were 8 cases of binaural nervous deafness,7 cases of binocular optic neuritis,and 6 cases of hepato-splenomegaly and/or lymphadenopathy.Amyloid A was significantly increased.Glucocorticoids and immunosup-pressive agents are the basic drugs for the treatment of this disease.If the curative effect was not good,biological agents should be added early to alleviate the disease.Conclusion CINCA syndrome is a rare autosomal dominant hereditary disease,the main clinical manifestations of which are skin,joint and central nervous system involvement,and even amyloidosis of organs.Early diagnosis and active treatment can reduce the involvement of important organs.

Clinical features analysis of osteonecrosis in 59 children with rheumatic diseases / 中华风湿病学杂志

Objective@#To analyze the clinical data of children with rheumatic diseases complicated with osteonecrosis and summarize the clinical characteristics, so as to guide clinical work.@*Methods@#The clinical data of 59 children with rheumatic diseases complicated with osteonecrosis from January 2010 to July 2018 were collected and analyzed retrospectively.@*Results@#Among 59 children with rheumatic diseases complicated with bone infarction, 25 cases were systemic lupus erythematosus (SLE), 4 cases were mixed connective tissue disease, 6 cases were juvenile dermatomyositis, 1 case was Takayasu arteritis, 1 case was leukocy to clystic vasculitis, 13 cases were systemic onset juvenile idiopathic arthritis (SJIA), 1 case was polyarthritis, and 8 cases were juvenile ankylosing spondylitis. The median time from the onset of rheumatic diseases to osteonecrosis onset was 18 (7.00, 38.75) months. A total of 115 joints were involved in 59 children, the most common of which were bilateral hips and knees. Twenty-five were single joint involvement and 34 were multiple joints involvement. There were 37 cases (63%) with vasculitis, 9 cases (15%) with oralulcer, 5 cases (8%) with Raynaud's phenomenon, 31 cases (53%) with Cushing's face, 18 cases (31%) with kidney involvement, 25 cases (42%) with hypertension, and 12 cases (24%) with spinal compression frac- tures. According to statistics, 10 children with osteonecrosis occurred without glucocorticoid intake. The longest duration of glucocorticoid therapy was 13 years, and the average duration was about (27±35) months whensymptomatic osteonecrosis occurred. The median cumulative dose of prednisone was 381.9(209.77, 561.19) mg/kg.@*Conclusion@#SLE, SJIA and juvenile ankylosing spondylitis are the three most common rheumatic diseases in children with osteonecrosis. The locations of osteonecrosis are mostly the bilateral hips and knees. It is necessary to strengthen joint examination, physical examination and imaging screening for children with rheumatic diseases after 18 months of onset, so early detection, early treatment are the strategy to improve the prognosis of the diseases.

Simultaneous Determination of 6 Flavonoids in the Roots of Tetrastigma hemsleyanum by HPLC / 中国药房

OBJECTIVE： To establish a method for simultaneous determination of rutin， isoquercitrin， kaempferol-3-O- rutinoside， daunorubicin， quercetin and kaempferol in the roots of Tetrastigma hemsleyanum.  METHODS： HPLC method was adopted. The determination was performed on Alliance SilGreen C18 column with mobile phase consisted of 0.2％ phosphoric acid solution-acetonitrile solution （gradient elution） at the flow rate of 1.0 mL/min. The detection wavelength was 360 nm and the column temperature was at 35 ℃. The sample size was 15 μL. RESULTS： The linear range of rutin， isoquercitrin， kaempferol-3- O-rutinoside， daunorubicin， quercetin and kaempferol were 21.77-217.77， 12.37-123.75， 13.23-132.31， 4.63-46.30， 5.75-57.50， 3.36-33.66 μg/mL （all r＝0.999 9）， respectively. limit of detection of them were 0.217 8， 0.123 8， 0.066 2， 0.046 3， 0.191 7， 0.112 3 μg/mL， respectively. limit of quantitation of them were 0.435 6， 0.247 5， 0.165 4， 0.154 3， 0.575 0， 0.421 2 μg/mL， respectively. RSDs of precision （n＝6）， stability （24 h， n＝7） and reproducibility tests （n＝6） were lower than 3.20％. The average recoveries of them were 96.23％， 86.88％， 97.51％， 97.67％， 97.50％， 87.46％， RSDs were 1.85％， 1.90％， 1.84％， 1.87％， 1.25％， 2.01％ （n＝9）， respectively. CONCLUSIONS： The method is fast and simple， and could be applied for simultaneous determination of rutin， isoquercitrin， kaempferol-3-O-rutinoside， daunorubicin， quercetin and kaempferol in the roots of T. hemsleyanum.  

Pay attention to the diagnosis and treatment of systemic-onset juvenile idiopathic arthritis / 中华实用儿科临床杂志

There is no specific clinical manifestation and laboratory test,the diagnosis of systemic-onset juvenile idiopathic arthritis (SoJIA) becomes difficuh.Some patients complicate with macrophage activation syndrome,if they are not treated timely,the mortality will be very high.Some patients were poor response to drug therapy,the disease relapsed frequently.So,they should pay attention to this difficult and serious disease in children.Diagnosis and treatment of SoJIA will be mainly elaborated below to enhance the level of diagnosis and treatment,and to improve the long-term prognosis of SoJIA.

Analysis of blood lipid characteristics and related factors in early childhood systemic lupus erythematosus / 中华风湿病学杂志

Objective To explore the characteristics of lipid profile in children with systemic lupus erythematosus (SLE) and its risk factors.Methods A total of 225 untreated children with SLE were enrolled in this study.According to the values of low-density lipoprotein cholesterol (LDL-C),very low density lipoprotein cholesterol (VLDL-C),high-density lipoprotein cholesterol (HDL-C),totalglycerin (TG) and triglyceride (TG),they were grouped into LDL-C normal group and elevated group,VLDL-C normal group and elevated group,HDL-C normal group and lower group,TG normal group and elevated group,TC normal group and elevated group.We used x2 test,binary logistic regression and t-test to analyze the relationship between various lipid groups and clinical symptoms,laboratory tests,disease activity,hormones and complications.Results One hundred and eighty-five cases (82.22％) of children with SLE developed dyslipidemia,mainly manifested as increased LDL-C,VLDL-C,TG,TC levelsand decreased HDL-C level.There was a positive correlation between urea nitrogen and TG (r=0.257,P＜0.01),positive correlation with LDL-C (r=0.129,P＜0.05)and positive correlation with VLDL-C (r=0.225,P＜0.01).Albumin and TG (r=-0.464,P＜0.01),TC (r=-0.246,P＜0.01),LDL-C (r=-0.138,P＜0.05),VLDL-C (r=-0.426,P＜0.05) were negatively correlated.Urine protein level (2 h) was positively correlated with TG (r=0.257,P＜0.01).Systemic lupus erythematosus disease activity index (SLEDAI) was positively correlated with TG (r=0.597,P＜0.01),positively correlated with VLDL-C (r=0.565,P＜0.01) and negatively correlated with HDL-C (r=-0.324,P＜0.01).Complement C3 was negatively correlated with TG (r=-0.284,P＜0.01).The positive proportion of anti-dsDNA antibody [TG-increased group (58/89) was higher than TG normal group (70/136) (x2=4.1 16,P=0.042);TC normal group (112/186) was higher than TC-increased group (16/39) (x2=4.841,P=0.028);LDL-C elevated group (7/21) was higher than LDL-C normal group (121/204) (x2=5.240,P=0.022)].The proportion of coronary involvement events in the TG increased group (25/89) was higher than that in the TG positive group (17/136) (x2=8.612,P=0.003).The proportion of LN was higher in each dyslipidemia group [TG increased group (54/89) was higher than TG normal group (34/136) (x2=28.75,P＜0.01);TC increased group (23/39) was higher than TC normal group (65/186)(x2=7.816,P=0.005);LDL-C elevated group (14/21) was higher than LDL-C normal group (75/204) (x2=7,385,P=0.007);VLDL-C elevated group (12/17) was higher than the VLDL-C normal group (76/208) (x2=7.651,P=0.006)] (P＜0.05).Conclusion Children with SLE have significant dyslipidemia.A variety of factors are involved in children with SLE dyslipidemia.Dyslipidemia can cause multiple organ damage in children with SLE.

The protective effect of bioimpedance spectroscopy guided ultrafiltration on residual renal function in hemodialysis patients / 中华疾病控制杂志

Objective To observe the protective effect of bioimpedance spectroscopy guided ultrahltration on residual renal function in new hemodialysis patients.Methods Patients with end-stage renal disease recruited from January 2015 to June 2016,were randomly divided into experimental group and control group.And all the patients were followed up for 3 months.The ultrafiltration was guided by the bioimpedance spectroscopy analysis in the experimental group,while the ultrafiltration was based on the edema,blood pressure,symptoms of low blood pressure and the increase of weight during the hemodialysis interphase in the control group.The difference of residual renal function,24 hours urine volume and the incidence rate of adverse events between the two groups were collected.Results Compared with the control group,the urine volume(932.58 ± 230.16 ml vs 584.45 ± 137.76 ml,t =7.226,P ＜ 0.001) and residual renal function (RRF) (4.55 ± 0.90 ml/min vs 3.08 ±0.68 ml/min,t =7.300,P ＜0.001)in the experimental group were higher.The drop of RRF(3.14 ±2.05 ml/min vs 4.40 ±2.09 ml/min,t =-2.384,P =0.020) and urinary volume (452.58 ±456.96 ml vs 877.45 ± 452.45 ml,t =-3.679,P =0.001) were lower in the experimental group.While there was no significant difference in the incidence of adverse events between the two groups (t =2.081,P =0.084).Conclusions It is helpful for slowing down the decline of residual renal function by using the bioimpedance spectroscopy guided ultrafiltration.

Changes in natural killer cell subsets defined by CD11b and CD27 in spleen of septic mice / 中华麻醉学杂志

Objective To evaluate the changes in natural Killer (NK) cell subsets definetd by CD11b and CD27 in the spleen of septic mice.Methods A total of 168 pathogen-free healthy male C57BL/6 mice,weighing 20-30 g,aged 8-10 weeks,were divided into 2 groups (n=84 cach) using a random number table:sham operation group (Sham group) and sepsis group (Sep group).Sepsis was induced by cecal ligation and puncture in chloral hydrate-anesthetized mice.Thirty mice in each group were selected to assess the survival within 4 days after operation,and the survival rate was calculated.At 2,4,6,24,48 and 72 h after operation (T1-6),6 mice were randomly selected,and blood samples were taken from the eyeballs for determination of serum tumor necrosis factor-alpha and interleukin-10 concentrations by enzyme-linked immunosorbent assay.Six mice were randomly selected at T4-6 and sacrificed,and the spleens were removed for measurement of the percentage of NK cells and their subsets by flow cytometry.Results Compared with Sham group,the survival rate was significantly decreased at different time points,the serum necrosis factor-alpha concentration at T1-3,6 and serum interleukin-10 concentration at T3-6 were increased,the percentage of NK cells was decreased at T4-6,the percentage of CD27-CD11b+NK cells was decreased and the percentage of CD27+CD11b+ and CD27+CD11b-NK cells was increased at T5,the percentage of CD27-CD11b-NK ceils was decreased at T4,5,and the percentage of CD27-CD11b-NK cells was increased at T6 in Sep group (P＜0.05).Conclusion Changes in NK cell subsets defined by CD11b and CD27 in the spleen play an important role in the development of sepsis in mice.

The study of cause of early death and a matched study for the risk factors in patients undergoing maintenance hemodialysis / 中华危重病急救医学

ObjectiveTo explore the cause of early death (death within 3-12 months after hemodialysis) and the related influencing factors patients undergoing maintenance hemodialysis (MHD) as to provide a scientific basis for the prevention of early death.Methods A retrospective matched controlled study was conducted. Fifty-one patients who underwent MHD from January 2004 to April 2014 and died within 3-12 months after hemodialysis in hemodialysis center of the 174th Chinese People's Liberation Army Hospital were included in the case group by retrospective analysis method. According to 1∶2 matched controls, 102 patients underwent hemodialysis in the same period (±2 months) and survived over 12 months were selected as control group. All patients received regular hemodialysis (dialysis 2-3 times per week), with conventional limitation of water and sodium intake, routine treatments such as control of blood pressure, treatment of anemia and disorders of calcium and phosphorus contents. Causes of short-term death were analyzed. Clinical and biochemical parameters of two groups were collected when dialysis was started, and the single factor and multiple factors logistic regression was used to analyze the related risk factors when dialysis was started. Receiver operating characteristic curve (ROC) was plotted to evaluate the value of above parameters in predicting the early death in patents with MHD.Results The main causes of early death of 51 patients with MHD were mainly cardiovascular and cerebrovascular diseases (27 cases, 52.9%), and infections (15 cases, 29.4%). It was shown by single factor analysis that the age [odds ratio (OR) = 6.625, 95% confidence interval (95%CI) = 3.232-13.580,P = 0.000], diabetes (OR = 3.875, 95%CI = 0.654 - 10.622,P = 0.031), specialist intervention time before dialysis (OR = 0.349, 95%CI =0.287 - 0.572,P = 0.004), the emergence of cardiovascular and cerebrovascular events before dialysis (OR = 9.667, 95%CI = 4.632 - 20.174,P = 0.000), the first dialysis for emergency dialysis (OR = 3.875, 95%CI = 1.713 - 8.765, P = 0.005), blood albumin level (OR = 0.294, 95%CI = 0.068 - 0.550,P = 0.008), leukocyte count (OR = 6.286, 95%CI = 1.648 - 23.982,P = 0.026), neutrophil count (OR = 2.833, 95%CI = 1.630 - 4.923,P = 0.001) might be the factors correlating with early death. Eight independent factors were statistically significant, and their effect on the MHD patients was analyzed by logistic regression analysis inα = 0.05 level. The results showed that patients with old age (OR = 1.054, 95%CI = 1.019-1.090,P = 0.002), and the emergence of cardio-cerebrovascular events (OR = 7.469, 95%CI = 2.474 - 22.545,P = 0.000)were early death risk factors of MHD patients, and early specialist intervention before dialysis was a protective factor (OR = 0.286, 95%CI = 0.113-0.722,P = 0.008). ROC curve showed that age had moderate diagnostic value for early death of MHD [area under ROC curve (AUC) = 0.756], the cut-off value was 59.0 years old, the sensitivity was 66.7%, and the specificity was 77.5%. The diagnostic value of early specialist intervention before dialysis was relatively low (AUC = 0.367), the cut-off value was 0.875 years, the sensitivity was 39.2%, and the specificity was 33.3%.Conclusion Old age, the emergency of cardiovascular and cerebrovascular events before dialysis is associated with early death, and specialist intervention ahead of dialysis can reduce the risk of early death.