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.

Clinical value of 2019 European League Against Rheumatism/American College of Rheumatology classification criteria in childhood-onset systemic lupus erythematosus / 中华实用儿科临床杂志

Objective:To compare the performance among the American College of Rheumatology (ACR) 1997, the Systemic Lupus International Collaborating Clinics (SLICC) 2012 and the 2019 European League Against Rheumatism (EULAR)/ACR criteria, in a childhood-onset systemic lupus erythematosus(CSLE) cohort.Methods:A medical chart review study was conducted of 182 cases of SLE patients and 163 controls with defined rheumatic diseases in pediatrics department of Renji Hospital, Shanghai Jiaotong University School Medicine, from January 2013 to May 2017, to establish each ACR1997, SLICC2012 and 2019EULAR/ACR criterion.The performance of the three criteria was statistically analyzed.Results:(1) Comparing the patients with SLE and controls, the difference in fever(21.4% vs.8.0%), skin lesions(54.9% vs.31.9%), nonscarring alopecia(3.8% vs.0), renal disorder(41.2% vs.5.5%), neurologic disorder(7.7% vs.1.8%), hematologic disorder [leukopenia(32.4% vs.1.8%), thrombocytopenia(31.9% vs.0)], low complement(83.5% vs.12.9%), anti-nuclear antibody(98.4% vs.23.3%), anti-dsDNA antibody(94.0% vs.8.6%), anti-Sm antibody(19.2% vs.0%), and antiphospholipid antibodies(16.5% vs.3.7%)had statistical significance (all P<0.05). But the difference in oral ulcers, synovitis, serositis and positive Coombs test had no statistical significance (all P>0.05). (2) Sensitivities of ACR1997, SLICC2012 and 2019EULAR/ACR criteria were 67.0%(122/182 cases), 95.6% (174/182 cases)and 97.8% (178/182 cases)( P<0.001), with specificities 99.4%(162/163 cases), 98.2% (160/163 cases)and 94.5%(154/163 cases) ( P=0.016), respectively.In terms of accuracy, the three classifications were 82.3%(284/345 cases), 96.8% (334/345 cases)and 96.2%(332/345 cases), respectively, the difference was statistically significant ( P<0.001). (3) Only 120 cases (65.9%) of patients with SLE met all 3 criteria.Eight cases of SLE patients who only met the 2019EULAR/ACR criteria exhibit high rate of single organ involvement (7 cases). Four cases of SLE patients were missed by the 2019EULAR/ACR, 3 cases of which were antinuclear antibody negative.(4) The SLICC2012 and 2019EULAR/ACR criteria had increased sensitivity for major organ damage than ACR1997.The total score of 2019 EULAR/ACR criteria correlated positively with SLE disease activity ( R2= 0.451, P<0.001). Conclusions:In this SLE population, the 2019EULAR/ACR criteria is more sensitive than ACR1997 and SLICC2012 criteria, allowing earlier classification and recognition of patients with single or major organ damage.Although the specificity is slightly lower than the previous two criteria, it is still worthy of clinical promotion.

Clinical features and factors associated with osteonecrosis in childhood-onset systemic lupus erythematosus / 中华实用儿科临床杂志

Objective@#To analyze the clinical features and factors associated with osteonecrosis in children with systemic lupus erythematosus (SLE).@*Methods@#A retrospective analysis of 15 SLE patients with osteonecrosis in Department of Pediatrics, Renji Hospital Affiliated to School of Medicine of Shanghai Jiaotong University from January 2013 to May 2017 was carried out.Forty-two SLE patients without osteonecrosis were selected as control group.The clinical, laboratory variables and the treatment were compared among SLE patients who were with and without osteonecrosis.@*Results@#(1) Fifteen patients developed osteonecrosis that constituted 8.6% of all the 175 hospitalized SLE patients during the same period.(2) Of 15 patients, 2 patients were male, 13 patients were female, who developed osteonecrosis with an average age of (13.9±2.7) years (range: 10-18 years old). The duration of SLE before the diagnosis of osteonecrosis ranged from 6 days to 141 months, the median was 10 months, and 80.0% (12/15 cases) was diagnosed with osteonecrosis within 2 years of SLE diagnosis.There were 36 joints involved in 15 patients, all of which were detected by magnetic resonance imaging(MRI). The knees were the most commonly involved joints(14/15 cases, 93.3%), followed by hip and ankle joints.(3) Univariate analysis revealed that the level of Triglyceride [(2.080±1.500) mmol/L vs.(1.350±0.945) mmol/L], maximum daily dose of glucocorticoid[(1.25±0.33) mg/kg vs.(1.07±0.22) mg/kg], positive rate of gene associated with glucocorticoid-induced osteonecrosis of femoral head(100.0% vs.54.8%)were significantly higher in SLE with osteonecrosis than those in controls(all P<0.05). While the level of 25(OH)D3[(21.37±11.29) μg/L vs.(31.45±17.73) μg/L] was significantly lower than that of controls(P<0.05). Multiple factor Logistic regression analysis showed that hypertriglyceridemia and daily maximum dose of glucocorticoid were the risk factors for osteonecrosis.@*Conclusions@#Osteonecrosis mostly occurred in children over 10 years old, knee joint involvement is the most common.The high-risk time of osteonecrosis is within 2 years of SLE diagnosis.Hypertriglyceridemia and daily ma-ximum dose of glucocorticoid are risk factors associated with osteonecrosis in children with SLE.

Clinical features and factors associated with osteonecrosis in childhood-onset systemic lupus erythematosus / 中华实用儿科临床杂志

Objective To analyze the clinical features and factors associated with osteonecrosis in children with systemic lupus erythematosus (SLE).Methods A retrospective analysis of 15 SLE patients with osteonecrosis in Department of Pediatrics,Renji Hospital Affiliated to School of Medicine of Shanghai Jiaotong University from January 2013 to May 2017 was carried out.Forty-two SLE patients without osteonecrosis were selected as control group.The clinical,laboratory variables and the treatment were compared among SLE patients who were with and without osteonecrosis.Results (1) Fifteen patients developed osteonecrosis that constituted 8.6％ of all the 175 hospitalized SLE patients during the same period.(2) Of 15 patients,2 patients were male,13 patients were female,who developed osteonecrosis with an average age of (13.9 ± 2.7) years (range:10-18 years old).The duration of SLE before the diagnosis of osteonecrosis ranged from 6 days to 141 months,the median was 10 months,and 80.0％ (12/15 cases) was diagnosed with osteonecrosis within 2 years of SLE diagnosis.There were 36 joints involved in 15 patients,all of which were detected by magnetic resonance imaging(MRI).The knees were the most commonly involved joints(14/15 cases,93.3％),followed by hip and ankle joints.(3) Univariate analysis revealed that the level of Triglyceride [(2.080 ± 1.500) mmol/L vs.(1.350 ± 0.945) mmol/L],maximum daily dose of glucocorticoid [(1.25 ± 0.33) mg/kg vs.(1.07 ± 0.22) mg/kg],positive rate of gene associated with glucocorticoid-induced osteonecrosis of femoral head (100.0％ vs.54.8％) were significantly higher in SLE with osteonecrosis than those in controls (all P ＜ 0.05).While the level of 25 (OH) D3 [(21.37 ± 11.29) μg/L vs.(31.45 ± 17.73) μg/L] was significantly lower than that of controls(P ＜ 0.05).Multiple factor Logistic regression analysis showed that hypertriglyceridemia and daily maximum dose of glucocorticoid were the risk factors for osteonecrosis.Conclusions Osteonecrosis mostly occurred in children over 10 years old,knee joint involvement is the most common.The high-risk time of osteonecrosis is within 2 years of SLE diagnosis.Hypertriglyceridemia and daily ma-ximum dose of glucocorticoid are risk factors associated with osteonecrosis in children with SLE.

Serum amyloid A protein in identification of disease activity and infection for systemic-onset juvenile idiopathic arthritis / 中华全科医师杂志

We retrospectively analyzed 126 children with juvenile idiopathic arthritis (JIA)admitted from January to December 2017,including 65 cases of systemic-onset JIA (SoJIA) and 61 cases of other types of JIA.The value of serum amyloid A protein (SAA) in the identification of the disease activity and infection in children with SoJIA was assessed.The area under the ROC curve of SAA in identification of disease activation of SoJIA patients was 0.934,which was not significantly different with other types of JIA.With the cut-off value of 68.32 mg/L the sensitivity and specificity for diagnosis of SoJIA activity were 0.913 and 0.892 respectively.In SoJIA patients the SAA was closely correlated with ESR and CRP (r=0.721 and 0.699,P＜0.001).The SAA level was significantly higher in the disease active stage than that in stable stage,in the stable stage with infection than that in the stable stage without infection of SoJIA patients.There were also significant differences in platelet and CRP between active disease with infection and active disease without infection.SAA is expected to be used for assessing disease activity of SoJIA,if combined with platelet and CRP,it may be of value in the identification of the complicating infection.

To strengthen the recognition to children arthritis related infection / 中华实用儿科临床杂志

Children arthritis associated infection can be divided into children infectious (invasive) arthritis and post infectious arthritis.Infectious arthritis onset with direct effects on the joints by pathogens,such as bacteria,fungi,viruses,tuberculosis infection.Post infectious arthritis onset often secondary to infection after infection factors of arthritis due to autoimmune reaction,such as reactive arthritis,arthritis after streptococcus infection,and so on.There are their clinical characteristics respectively in infectious arthritis and post infection arthritis in children,and it is important for the diagnosis,treatment and prognosis of children with arthritis to be familiarity with their characteristics.In addition,the factor of infection is closely related to juvenile idiopathic arthritis.It should be pay attention to the role of infectious factors in triggering and aggravating the disease.

Case-control study and Logistic analysis of the risk factors of infants with recurrent wheezing in Shanghai Pujiang area / 中华实用儿科临床杂志

Objective To analyze the relevant risk factors of recurrent wheezing(≥3 attacks) in the first 3 years of life in Shanghai Pujiang.Methods A case-control study was conducted.Two hundred and sixty-two research children were chosen for clinical visits (＜ 3 years old) with wheezing at the Pediatric Department of Shanghai Renji Hospital (South Campus),School of Medicine,Shanghai Jiaotong University,from January to December 2014.According to the frequency of wheezing,the subjects were divided into 75 cases of recurrent wheezing group (≥ 3 attacks),110 cases of occasional wheezing group(1-2 attacks) and 77 cases of no wheezing group.Probable risk factors were inquired by using face-to-face questionnaire.The passive agglutination method was used to detect the Mycoplasma pneumoniae antibody immunoglobulin M (IgM).The indirect immunofluorescence was used to detect the respiratory pathogens.The Western blot was used to detect 20 items of serum allergen.Chi-square test was firstly used for univariate analysis,and then the multivariate stepwise Logistic regression was used to analyze the independent risk factors associated with infant recurrent wheezing.Results A total of 20 factors were found relevant to infant recurrent wheezing by univariate analysis,which included boys (OR =4.030,95％ CI:1.937-8.388),personal atopy (OR =13.125,95％ CI:5.951-28.946),allergic dermatitis (OR =9.833,95％ CI:4.663-20.737),allergic rhinitis (OR =40.327,95％ CI:5.300-306.842),like rubbing eyes or nose(OR =6.487,95％ CI:3.190-13.191),food allergy (OR =6.689,95 ％ CI:1.860-24.051),premature birth (OR =3.795,95 ％ CI:1.001-14.385),low birth weight (OR =9.075,95％ CI:1.106-74.450),parental atopy (OR =10.667,95％ CI:4.824-23.587),parental allergic dermatitis (OR =8.072,95 ％ CI:2.634-24.734),parental allergic rhinitis (OR =6.524,95 ％ CI:2.920-14.577),parental allergic conjunctivitis (OR =1.087,95％ CI:1.017-1.162),parental asthma history (OR =1.119,95％ CI:1.035-1.210),colds ＞ 6 times (OR =9.111,95％ CI:3.970-20.909),history of bronchopneumonia(OR =7.554,95％ CI:3.588-15.903),age at first time use of antibiotics less than 6 months (OR =2.388,95％ CI:1.129-5.052),exposure to cigarette smoking (OR =1.922,95 ％ CI:1.004-3.681),maternal passive smoking during pregnancy (OR =2.508,95 ％ CI:1.298-4.848),living close to wood stove (OR =3.342,95 ％ CI:1.427-7.827) and positive results of inhaled allergens (OR =1.821,95 ％ CI:1.420-2.336).Keeping cats was the protective factor(OR =0.922,95％ CI:0.864-0.984).The forward Logistic regression analysis showed that personal atopy (OR =10.278,95 ％ CI:2.503-42.202),like rubbing eyes or nose (OR =1 0.316,95 ％ CI:2.722-39.101),food allergy (OR =10.370,95％ CI:1.248-86.145),parental atopy (OR =5.402,95％ CI:1.340-21.778),colds ＞ 6 times (OR =7.048,95 ％ CI:1.688-29.423),history of bronchopneumonia (OR =7.876,95 ％ CI:2.040-30.407) and maternaal passive smoking (OR =3.696,95 ％ CI:1.013-13.494) during pregnancy were the independent risk factors of infants recurrent wheezing.Conclusion Personal atopy,like rubbing eyes or nose,food allergy,parental atopy,colds ＞ 6 times,history of bronchopneumonia,maternal passive smoking are the independent risk factors of recurrent wheezing in infants less than 3 years old.

Influence of atopy on prognosis of juvenile-onset systemic lupus erythematosus / 中华实用儿科临床杂志

Objective To evaluate the influence of atopy on the prognosis of juvenile-onset systemic lupus erythematosus (JSLE).Methods The study was performed on 60 cases with JSLE diagnosed at the Department of Pediatrics,Renji Hospital Affiliated to School of Medicine of Shanghai Jiaotong University from October 2005 to April 2015.These patients were enrolled by mixed cohort study and subdivided into atopic group(26 cases) or non-atopic group(34 cases).The clinical and laboratory data of the disease onset,disease assessment scores,medications during follow-ups and remission/flare of the disease were recorded and analyzed to compare the difference between 2 groups.Results (1) The systemic lupus erythematosus disease activity index (SLEDAI) score [(17.080 ± 5.628) scores vs (12.590 ± 4.856) scores],anti-double-stranded DNA (anti-dsDNA) [(62.590 ± 43.602) IU/mL vs (40.230 ±30.189) IU/mL],erythrocyte sedimentation rate (ESR) [(59.150 ± 40.315) mm/1 h vs (40,350 ± 31.865)mm/1 h] were significantly elevated at onset in the atopic group compared with non-atopic controls (all P ＜ 0.05),while the complement C3[(0.450 ±0.218) g/L vs(0.640 ±0.333) g/L],C4 [(0.047 ±0.024) g/L vs(0.116 ±0.172) g/L] in atopic group was lower than those in the non-atopic group (all P ＜ 0.05).(2)During the follow ups of 1 and 6 months to 1 year,the JSLE patients with atopy always had higher SLEDA1 score compared with the non atopic controls(all P ＜ 0.05).(3)For medications,the daily cumulative glucocorticoid dose received by patients in the atopic group were larger than that of the non-atopic group,and the number of immunosuppressive agents used in the atopic group was more than that in the non-atopic controls (P ＜ 0.05).(4) During the 1-year follow-up,the rate of disease relapse in the atopic group was higher than that in the non-atopic group and the atopic group also needed much more time to reach disease remission (P ＜ 0.05).Conclusion JSLE patients combined with atopy may have an adverse influence on the prognosis of JSLE.

Meta analysis of the risk factors of recurrent wheezing in infants / 中华实用儿科临床杂志

Obgective To analyze the relevant risk factors of recurrent wheezing(≥3 attacks) in the first 3 years of life.Methods Wheezing,respiratory sounds,risk factor were used as key words to retrieve papers in Chinese literature databases including Sinomed,Wanfang and Weipu databases.The same strategy was used to retrieve English papers in English literature databases including PubMed,Cochrane library and Embase.Time range was from 31th May 2004 to 1 rd June 2014.The execution of quality evaluation of the included documents was in compliance with Newcastle-Ottawa Scale and cross-sectional study standard recommended by Agency for Healthcare Research and Quality.The evidence quality evaluation was conducted with GRADEpro and followed by the Meta analysis with RevMan 5.2.R~ults A total of 13 studies were included in this Meta-analysis.Several factors were related to recurrent wheezing episodes,including risk factors such as maternal smoking during pregnancy (OR =1.47,95％ CI:1.30-1.66),asthma in parents (OR =1.94,95 ％ CI:1.72-2.19),family history of atopy (OR =1.94,95％ CI:1.72-2.19),male (OR =1.42,95 ％ CI:1.19-1.69),history of eczema (OR =2.36,95 ％ CI:1.69-3.30),colds (＞ 6 times) (OR =2.02,95 ％ CI:1.54-2.64),history of bronchopneumonia (OR =1.85,95 ％ CI:1.46-2.34),exposure to cigarette smoking(OR =2.30,95％ CI:1.68-3.14),daycare attendance(OR =2.27,95％ CI:1.97-2.60);Education received by the mother ＞ 12 years (OR =0.80,95％ CI:0.70-0.92) was the protective factor.Conclusions The risk factors of recurrent wheezing(≥3 attacks) in the first 3 years of life are maternal smoking during pregnancy,asthma in parents,family history of atopy,male,history of eczema,colds (＞ 6 times),history of bronchopneumonia,exposure to cigarette smoking and daycare attendance.The protective factor is education received by the mother ≥ 12 years.The prerequisite in precaution of infants recurrent wheezing is to ensure the utmost avoidance of hazardous factors and reinforcement of protective factors.

Immune tolerance in the pathogenesis of juvenile idiopathic arthritis / 国际儿科学杂志

Juvenile idiopathic arthritis is a disease characterized by autoimmune disorders and immune imbalance. In recent years,immune tolerance has become a hot issue in the pathogenesis of juvenile idiopathic ar-thritis. In this article,cytotoxic T lymphocyte associated-antigen 4,programmed death-1,Treg cells and apoptotic cells in the pathogenesis of juvenile idiopathic arthritis are summarized.

T helper lymphocyte subsets and cytokines in children with systemic-onset juvenile idiopathic arthritis / 临床儿科杂志

Objectives To explore the changes of T helper (Th) lymphocyte and its related factors in children with syste-mic-onset juvenile idiopathic arthritis (SoJIA). Methods A total of 36 SoJIA inpatients, hospitalized from January 2012 to June 2013, were divided into active phase group and remission group. In addition, 20 healthy children were selected as normal con-trols. Th1, Th2 and Th17 cell ratios in peripheral blood mononuclear cells were detected and compared between each group by flow cytometry. Serum interferon-γ(INF-γ), interleukin-4 (IL-4), interleukin-17 (IL-17) levels were measured by enzyme-linked immunosorbent assay. Results The proportions of Th17 cells over CD3+CD8-cell were (3.30±2.15)%, (1.78±1.14)%and (1.22± 1.14)%in active phase group, remission group and control group. The difference among three groups was significant (H=14.437, P=0.001), and the active phase group had higher proportion of Th17 than the other two groups (P0.05). The serum IL-17 levels were (125.82 ± 45.87) pg/ml, (57.79±25.84)pg/ml and(50.02±18.37)pg/ml in active phase group, remission group and control group with signifi-cant difference among three groups (F=31.82, P=0.000), and the active phase group had higher level of IL-17 than the other two groups (P0.05). Conclusions Acquired cellular immunity is involved in pathogenesis of SoJIA, the increased proportion of Th1 and Th17 cell and the changes of related cytokines seem to correlate with active phage of SoJIA.

2013 update of the 2011 American College of Rheumatology recommendations for the treatment of juvenile idiopathic arthritis (selected translation) / 中华实用儿科临床杂志

The American College of Rheumatology (ACR) updated the 2011 recommendations for juvenile idiopathic arthritis (JIA) in 2013 and focused on the treatment of systemic-onset juvenile idiopathic arthritis (SOJIA).According to the clinical feature of the SOJIA,the subtype is developed to 3 phenotypes:(1) phenotype of systemic JIA with active systemic features and yarying degrees of synovitis; (2) phenotype of systemic JIA without active systemic features and with varying degrees of active synovitis ; (3) phenotype of systemic JIA with features concerning for macrophage activation syndrome (MAS).The treatment recommendation was made according each phenotype,including initial therapeutic options and therapeutic options for continued disease activity.And uncertain or inappropriate options for continued disease were listed alphabetically.Development of treatment recommendations for children with SOJIA and features of MAS is particularly challenging.However,it is anticipated that in the near future,with research of the disease process,the better recommendation may be made.

Interperting update of the 2011 American College of Rheumatology recommendations for the treatment of juvenile idiopathic arthritis in 2013 / 中华实用儿科临床杂志

The American College of Rheumatology (ACR) published treatment recommendations for juvenile idiopathic arthritis (JIA) in 2011.The recommendations meant as a guide to health care providers caring for JIA children with dividing JIA into 5 distinct subtypes.The ACR updated the 2011 recommendations in 2013 and focused on the treatment of systemic-onset juvenile idiopathic arthritis (SOJIA).According to the clinical feature of the SOJIA,the subtybe is developed to 3 phenotypes.And the conception was firstly addressed that systemic JIA with features concerning for macrophage activation syndrome(MAS).Different treatment options was applied for clinical manifestations score,and these recommendations offered guidance for providers caring for children with the most common phenotypes associated with systemic JIA,rather than exceptional cases with unusual disease manifestations or refractory disease.

The influence of allergic rhinitis on patients with systemic onset juvenile idiopathic arthritis / 中华风湿病学杂志

Objective To explore the influence of allergic rhinitis (AR) on patients with systemic onset juvenile idiopathic arthritis (SoJIA).Methods The study involved 44 cases with SoJIA from Department of Pediatrics,Renji Hospital affiliated to School of Medicine of Shanghai Jiaotong University from July 2008 to November 2013.The Clinical and laboratory data of all patients were recorded respectively.This was a retrospective cohort study.According to the diagnosis of allergic rhinitis (AR),children were subdivided into AR group (16 cases) and Non-AR group (28 cases).ACR Pediatric criteria (ACR Pedi) 30/ 50/70 and related indicators of SoJIA between the two groups were compared.In the AR group,the correlation between AR scores and DAS28 was analyzed.When SoJIA of the two groups relapsed,the AR group (the treatment group) received anti-rheumatism for arthritis as well as nasal spray and oral antihistamines for AR.The non-AR group (control group) only received the anti-rheumatism for arthritis.The improvement of SoJIA between the two groups was analyzed.The continuous variables were analyzed by Student's t-test or the MannWhitney U-test as appropriate.Categorical data were compared between different groups by the Chi-square test.Correlations were determined by Pearson or Spearman's ranking.Results ① In the retrospective analysis:the physician's and patients'/parents' general assessment on a 10 cm visual-analogue scale (VAS),number of joints with res-triction of movement,number of swollen joints,ESR,serum ferritin(SF) and childhood health assessment questionnaire (CHAQ) score were significantly elevated in the AR group compared with Non-AR group at the disease onset [(6.7±1.0) cm vs (4.8±1.9) cm; (6.5±1.4) cm vs (3.2±1.5) cm; 4.1±2.7 vs 2.7± 1.7; 3.4±2.4 vs 1.4±1.5; (87±35) mm/1 h vs (61±40) mm/1 h; (888±1 043) μg/L vs (311±324) μg/L; 1.6±0.5 vs 0.7±0.3,respectively; all P＜0.05].At the 3 and 6 months follow-up after disease onset,the proportion of patients who reached ACR pedi 50,70 in AR group were lower than the Non-AR group,while the cumulative glucocorticoid dose was higher in the AR group than that of those without AR [at 3 months 38％ vs 71％; 13％ vs 46％; (76±35) mg/kg vs (43±36) mg/kg,respectively; at 6 months 25％ vs 71％; 19％ vs 64％; 127±57 vs 67±58,respectively,all P＜0.05]; In the AR group,at the disease onset,3 and 6 months follow-up after disease onset,the scores of AR was positively correlated with DAS28(r=0.741,0.703,0.680,respectively; all P＜0.05).② In the prospective study:when SoJIA was relapsed,systemic feature score,the physician's and patients' /parents' general assessment on a l0 cm VAS,number of joints with restriction of movement,number of swollen joints,ESR,SF and CHAQ score were significantly elevated in the treatment group compared with the control group [3.8±1.5 vs 2.1±1.1; (5.6±1.5) cm vs (4.5±1.6) cm; (4.6±1.9) cm vs (3.1±1.5)cm; 3.9±1.9 vs 2.5±1.4; 2.4±0.9 vs 1.5±1.2; 92±27 vs 53±37; 565(339,1 192) μg/L vs 171(85,284) μg/L; 13(0.8,1.6) vs 0.7(0.5,1); respectively; P＜0.05].The improvement rate of the physician's and patients'/parents' general assessment on a 10 cm VAS,number of swollen joints,number of joints with restriction of movement,ESR and CHAQ score at the follow-up 3 months were higher in treatment group than the control group [71(55,86)％ vs 46(0,75)％; 67(45,81)％ vs 28(-4,62)％; 92(77,96)％ vs 70(27,88)％; 65(48,81)％ vs 0(-17,67)％; 100(46,100)％ vs 42(0,100)％; 67(49,85)％ vs 37(0,75)％; P＜0.05].At the follow-up 6 months,the improvement rate of ESR,patients'/ parents' general assessment on a 10 cm VAS,number of joints with restriction of movement and CHAQ score were higher than control group [94(85,96)％ vs 73(33,85)％; 89(65,99)％ vs 63(5,85)％; 100(100,100)％ vs 100(0,100)％; 91(69,100)％ vs 72(11,91)％; respectively,P＜0.05].Conclusion AR may exert an adverse influence on SoJIA.SoJIA patients who are treated with combined with AR may have better outcome than those who are only treated for arthritis.

Influence of atopy on juvenile idiopathic arthritis / 中华实用儿科临床杂志

Objective To evaluate the inlluence of atopy on juvenile idiopathic arthritis (JIA).Methods The study involved 117 cases with JIA from Department of Pediatrics,Renji Hospital Affiliated to School of Medicine of Shanghai Jiaotong University from Jul.2008 to Jul.2013.These patients were enrolled for retrospective cohort study,and subdivided into JIA and atopic group or JIA and non-atopic group.There were 34 cases combined with atopy,83 cases without atopy.Based on the diagnosis of allergic rhinitis (AR),those JIA children in the atopic group were organized into AR group (19 cases) and non-AR group (15 cases).The clinical and laboratory data were recorded and analyzed to compare the differences of the remission of American College of Rheumatology Pediatric (ACR Pedi) 30/50/70 between atopic group and non-atopic group,AR group and non-AR group.In AR group,the correlation between AR scores and disease activity score (DAS28) was analyzed.Results 1.The physician's and patients'/parents' general assessment on a 100 mm visual-analogue scale (VAS),number of joints with restriction of movement and childhood health assessment questionnaire (CHAQ) were significantly elevated in atopic group compared with controls at the beginning (all P ＜ 0.05).In the follow-up 3 months after disease onset,the proportion of reaching ACR Pedi 30,50 and the proportion of reaching ACR Pedi 50,70 in 6 months later in JIA with atopy were lower than JIA children without atopy (all P ＜ 0.05) ; In the follow-up 3 and 6 months,the cumulative glucocorticoid dose was higher in atopy group compared with Non-atopy,which showed a statistical significance (all P ＜ 0.05).2.Among the AR group,at the disease onset,the physician's and patients'/parents' VAS,number of joints with restriction of movement and CHAQ were elevated in AR group compared with controls with statistical significance (all P ＜ 0.05).In the follow-up 3 months,the proportion of reaching ACR Pedi 30 and 50 was lower in AR group compared with non-AR group.In the follow-up 6 months,the cumulative glucocorticoid dose was higher in AR group compared with non-AR,which showed a statistical significance.But the ratio of ACR Pedi 30,50 and 70 were lower in AR group compared with non-AR group (all P ＜ 0.05).Among JIA combined with AR,at the beginning,follow-up 3 and 6 months after disease onset,the scores of AR positively correlated with DAS28 (r =0.671,0.518,0.496,all P ＜ 0.05).Conclusion Atopy or AR may exert an adverse influence on JIA.

Preliminary identification of children with acute lymphoblastic leukemia and juvenile idiopathic arthritis / 国际儿科学杂志

With acute lymphoblastic leukemia in skeletal muscle symptom,part of these patients may be misdiagnosed as juvenile idiopathic arthritis. How to distinguish these children has significance for the timely and proper treatment and good prognosis. This article from the history and routine laboratory examination and ima-ging examination put forward early in the disease through the analysis of joint symptoms from juvenile joint pain,blood and imaging characteristics and preliminary identification of childhood leukemia from juvenile idio-pathic arthritis in order to decrease the rate of misdiagnosis.

Clinical value of common allergens in children with bronchial asthma in Jiading area of Shanghai / 国际儿科学杂志

Objective To investigate the common allergens and the relationship between the common allergens and the age of children with bronchial asthma in Jiading area of Shanghai. Methods Skin prick test (SPT) of 15 common allergens with standard prick liquid were performed in 351 asthmatic children, the children were divided into ≤3 years group, 3 ～6 years group and ＞6 years group, and we observed the positive rates and allergens in different age groups. Results (1) The positive rate of SPT for inhalant allergens in children with bronchial asthma is 71. 2%, specifically are: dust mite (49. 6%), house dust mite (49. 0%), fungi Ⅰ (36. 8%), mold Ⅱ (34. 8%), tree Ⅰ (32. 5%), dog epithelium (31.9%), cat epithelium (31.3%), pollen (29. 1%), tree Ⅱ (28. 8%), and feather (27.4%). (2)The positive rate of SPT for food allergens in children with bronchial asthma is 39. 6%, specifically are: shrimp(24.2%), eggs(15.7%), milk(14.8%), peanuts (13. 7%), and curries(12.5%). (3) The positive rate of inhalant allergens was significantly higher than food allergens. The positive rate of inhalant allergens had no significant differences among different ages, while the positive rate of food allergens was increased with age. Conclusions Most children with bronchial asthma and allergens are related, and allergens are related to age.

Five cases of childhood primary Sjogren's syndrome and literature review / 中华风湿病学杂志

ObjectiveTo further understand the clinic manifestations of childhood primary Sjogren's Syndrome(pSS) and enhance early diagnosis. MethodsFive cases of pSS from Renji Hospital, Shanghai, were reported and their clinical features were analysoed. And literatures from Medline database and Weipu database were reviewed and discussed. Results①Childhood pSS had various clinic presentations that were non-specific and sicca symptoms were absent or occur late in most cases. ② The most common presentations were recurrent parotiditis and cutaneous manifestations with various locations and forms. ③ American-European Criteria for SS were not suitable for the diagnosis of childhood pSS. ConclusionRecurrent parotiditis and cutaneous manifestations in children can be used as clues for the diagnosis of childhood pSS but needs to be further confirmed by the positive results of salivary gland biopsy and autoantibodies examination, particularly SSA/SSB.

Analysis of the treatment response and prognosis of severe hand-foot-mouth disease / 中华传染病杂志

Objective To investigate the significance of early diagnosis and intervention in cases with severe hand-foot-mouth disease. Methods Nine severe cases were chosen from 220 hospitalized children with hand-foot-mouth disease for retrospective analysis, including onset, disease progression, the blood and cerebrospinal fluid tests, electroencephalogram data, patients' treatment responses and prognosis. Pearson X2 test and t test were utilized for statistical analysis. Results All cases showed nervous systems involved symptom, including meningeal irritation sign and (or) other pathological signs of nervous system (9 cases), drowsy (7 cases), trembling (6 cases), voiding dysfunction (3 cases), hypersensitivity(3 cases), autonomic nervous system disorders (2 cases), ataxia (1 case), left leg mild paralysis (1 case) and early stage of pulmonary edema (1 case). Early intervention, such as high dose gamma globulin, methylprednisolone, mannitol treatment restriction of fluid input, started before the development of heart and lung failure. No case died but one patient with encephalomyelitis showed hobbling left leg, which didn't recover until 6 weeks later. One case with brainstem encephalitis still showed abnormal electroencephalogram after 8 weeks follow-up but without clinical symptom. Conclusions Enterovirus 71 can cause severe hand-foot-mouth disease complicated by encephalitis, meningitis and pulmonary edema. Early active intervention before the development of neurogenic pulmonary edema can improve the prognosis and reduce the mortality.