Reactive arthritis(ReA) is a kind of sterile, non-purulent arthritis that occurs after microbial infections far from the joints.The disease has a broad spectrum, and it can be classified into three categories based on clinical characteristics: human leukocyte antigen B27(HLA-B27)-associated ReA, acute rheumatic fever(ARF), and post-streptococcal reactive arthritis(PSRA).In addition to joints, it may also involve the gastrointestinal tract, skin, eyes, and heart.Unlike adults, the pathogenesis of ReA in children is more complex.HLA-B27-associated ReA is more common after gastrointestinal and respiratory infections, with less involvement of the central axis and sacroiliac joints and more involvement of the hip and peripheral joints and attachment point inflammation.ARF is most common in children aged 5 to 15 years, characterized by migratory and multiple arthritis.The duration of onset of PSRA in children is shorter than that in adults.This article reviews the epidemiology, clinical manifestations, diagnosis and treatment of ReA in children to improve clinicians′ understanding of ReA in children.

Juvenile idiopathic arthritis associated uveitis (JIA-U) is a primary extra-articular complication of juvenile idiopathic arthritis, which can cause symptoms such as red eyes, eye pain, photophobia, floating shadows in front of the eyes, or decreased vision.In severe cases, it can induce blindness and cause significant harm to children and their families.The American College of Rheumatology, the Multinational Interdisciplinary Working Group for Uveitis in Childhood, the Pediatric Rheumatology European Society and the Canadian Rheumatology Association have released relevant clinical guidelines and expert consensus on this disease.This article mainly interprets the above-mentioned clinical guidelines and expert consensus regarding the diagnosis, treatment, and long-term management of JIA-U, to improve the efficiency and management quality in clinically diagnosing and treating JIA-U.

Objective:To evaluate the relationship between the mechanism of propofol inhibiting carotid sinus baroreflex (CSR) and GluR2 subunit-containing AMPA receptors in the nucleus ambiguus of rats with type 2 diabetes mellitus (T2DM).Methods:SPF healthy male Sprague-Dawley rats, aged 3 weeks, were selected and fed a high glucose and high fat diet for 6 weeks, and then streptozotocin 30 mg/kg was intraperitoneally injected to prepare a T2DM model of rats. Twenty-four T2DM rats were divided into 4 groups ( n=6 each) using a random number table method: diabetes mellitus-normal saline group (DN group), diabetes mellitus-propofol group (DP group), AMPA receptor agonist-normal saline group (AN group), and AMPA receptor agonist-propofol group (AP group). Another 12 normal rats were selected and divided into 2 groups ( n=6 each) using a random number table method: normal-normal saline group (NN group) and normal-propofol group (NP group). AMPA receptor agonist mibamitor 1 nmol/L (50 nl) was injected into the nucleus ambiguus using a micropipette at 30 min before perfusion of isolated carotid sinus in AN and AP groups. Propofol 45 mg·kg -11·h -1 was infused for 2 h via the femoral vein in NP group, DP group and AP group, and the equal volume of normal saline was given instead in the other groups. A model for perfusing isolated carotid sinus was developed at 20 min after infusion of propofol or normal saline, the intracarotid sinus pressure (ISP)-mean arterial blood pressure (MAP) curve was drawn, and CSR parameters such as maximum slope (PS), threshold pressure (TP), saturation pressure (SP), equilibrium pressure (EP), maximum decrease in MAP reflexivity (RD), and carotid sinus baroreceptor operating range (OR) were recorded. Brain tissues were taken at the end of perfusion, and the expression of GluR2 subunit in the nucleus ambiguus was detected by Western blot and immunofluorescence. Results:Compared with the corresponding normal saline groups (NN group, DN group, AN group), PS and RD were significantly decreased, TP, SP and OR were increased ( P<0.05), and the ISP-MAP curve was shifted upward in propofol groups (NP group, DP group, AP group), the expression of GluR2 subunit in the nucleus ambiguus was down-regulated in NP and DP groups ( P<0.05), and no significant change was found in the expression of GluR2 subunit in the nucleus ambiguus in AP group ( P>0.05). Compared with NP group, PS and RD were significantly decreased, TP, SP and OR were increased ( P<0.05), the ISP-MAP curve was shifted upward, and the expression of GluR2 subunit in the nucleus ambiguus was down-regulated in DP group ( P<0.05). Compared with DP group, PS and RD were significantly increased, TP, SP and OR were decreased ( P<0.05), the ISP-MAP curve was shifted downward, and the expression of GluR2 subunit in the nucleus ambiguus was up-regulated in AP group ( P<0.05). Conclusions:The mechanism by which propofol inhibits CSR may be related to down-regulation of the expression of GluR2 subunits-containing AMPA receptors in the nucleus ambiguus of rats with T2DM.

  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.

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.

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.

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.

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.

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.

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.