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Objective:To investigate the clinical features, treatment and follow-up of children with early-onset antinuclear antibody (ANA)-positive juvenile idiopathic arthritis (JIA).Methods:Eighty-six oligoarticular JIA patients with early-onset arthritis (≤6 years old) admitted to the Children′s Hospital Affiliated to Capital Institute of Pediatrics from January 2017 to December 2019 were included in this study. According to ANA titer, these patients were divided into two groups: ANA-positive group (44 cases) and ANA-negative group (42 cases). Clinical data including demographic data, clinical features, laboratory testing results, treatment and follow-up data were statistically analyzed.Results:The ratio of male to female was 7∶37 in the ANA-positive group and 15∶27 in the ANA-negative group and there was significant difference between the two groups ( P=0.035). The proportions of patients with increased C-reactive protein and erythrocyte sedimentation rate were higher in the ANA-positive group than in the ANA-negative group [18.18% (8/44) vs 16.67% (7/42) and 29.55% (13/44) vs 19.05% (8/42), both P>0.05]. The most commonly involved joints in the ANA-positive group were knee (95.45%, 42/44), ankle (20.45%, 9/44) and wrist (18.18%, 8/44), and unilateral asymmetric joint involvement accounted for 81.8% (36/44). In the ANA-negative group, the involved joints were knee (85.71%, 36/42), ankle (14.29%, 6/42), wrist (14.29%, 6/42) and hip (11.90%, 5/42), and 27 out of the 42 cases (64.29%) had unilateral asymmetric joint involvement. There was no significant difference in the above indexes between the two groups (all P>0.05). There were seven cases (15.91%) with uveitis in the ANA-positive group and two cases (4.76%) in the ANA-negative group, and the difference between the two groups was significant ( P=0.045). Before treatment, the ANA-positive group had a significantly higher disease activity score (JADAS27) than the ANA-negative group (14.43±2.87 vs 12.09±3.32, P=0.002). After treatment, the JADAS27 score in both groups decreased (both P<0.05). After six months of treatment, the two groups had similar clinical remission rates [70.45% (31/44) vs 76.19% (32/42), P>0.05]. Conclusions:Early-onset ANA-positive JIA was more common in female children, and asymmetric knee joint involvement was the most common clinical manifestation. The incidence of ophthalmic complications was high, and ophthalmological examination should be performed more frequently during follow-up. The prognosis of early-onset ANA-positive JIA was good with early treatment. Positive ANA was not a risk factor for poor prognosis.
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Objective:To analyze the clinical characteristics of infantile Takayasu Arteritis (TAK) complicated with cardiac involvements.Methods:The clinical data and cardiac lesions of infantile TAK were collected retrospectively, and the clinical characteristics of the disease were analyzed and summarized. Mainly using decriptive statistical methods.Results:In these 20 cases, 16 cases (80%) had cardiac involvements, only 2 cases had related symptoms. The common lesions were coronary artery lesion (CAL), valvular disease, and elevated myocardial enzymes, while the rare lesions were arrhythmia, pericardial effusion, hypertensive heart disease, and heart failure. One case had acute heart failure, which was systolic heart failure and was accompanied by hypertensive heart disease. All 14 patients with CAL were found by conventional coronary ultrasound screening. A total of 39 CAL were found, all of which were coronary artery dilation, and the left main coronary artery was involved. Five patients had heart valve disease, all of them were valve insufficiency. The involved valves were mitral and tricuspid valves, and one of them was severe insufficiency. Arrhythmias were found in 2 cases, of which P1 was found to have paroxysmal atrial tachycardia with high atrioventricular block at 3 months. All 20 children survived and were in stable condition after being treat with biological agents and/or glucocorticoids. A case of hypertensive heart disease complicated with heart failure was followed up for 4 years, and the cardiac function and blood pressure returned to normal. Fourteen children with CAL lesions were given oral aspirin disease, the CALs disappeared in 10 cases and retracted in 4 cases. During the follow-up of 5 children with heart valves, insufficiency disappeared in 4 cases and improved in 1. No child underwent valve replacement during the follow-up. One of the children with arrhythmia was treated with antiarrhythmic drugs. After treatment, the arrhythmia disappeared. Now they have been followed up for 5 years without recurrence.Conclusion:Infantile TAK has a high incidence of heart involvement, with extensive lesions but insidious clinical symptoms. CALs are common, and heart failure is rare. It should be evaluated and treated as early as possible.
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Clinical data of a child with mevalonic aciduria (MA) who underwent stem cell transplantation (SCT) in the Department of Rheumatology and Immunology, Children′s Hospital, Capital Institute of Pediatrics in March 2019 were retrospectively analyzed.A girl aged 2 years and 11 months old presented with recurrent fever for 2 years and 11 months and swelling of both knees for 9 months was enrolled.The child also had specific facial features and development delay.The urinary mevalonic acid and inflammatory factor levels were increased.The whole exome sequencing showed compound heterozygous mutations c. 439G>A (p.A147T) and c. 976G>A(p.G326R) in the MVK gene.After achieving a partial remission following the treatment of Tocilizumab, the patient was treated with SCT and thus yielded the complete remission.Through literature review of a total of 39 children with MA, most of cases suffer MA since the infancy.All systems can be affected by MA.Clinical manifestations of the nervous system abnormalities, recurrent fever, hepatosplenomegaly, delayed physical development, gastrointestinal symptoms, and eye involvement were helpful for the diagnosis of MA.To date, 10 cases (including one case in this study) of MA have been reported to receive SCT after achieving a partial remission of other treatment, and 7 finally achieve a complete remission.This case report provided references that SCT is an effective treatment to children with MA who fail to achieve a complete remission after conventional treatment.
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Objective:To explore the clinical features, follow-up characteristics and prognosis of rheumatic disease complicated with pulmonary arterial hypertension (PAH) in children, and to provide support for its clinical diagnosis and treatment.Methods:A retrospective analysis was conducted on the data of rheumatic 24 patients complicated with PAH hospitalized in the Department of Rheumatology and Immunology, Children′s Hospital Affiliated to the Capital Institute of Pediatrics, Department of Rheumatology and Immunology, Jiangxi Children′s Hospital, Department of Pediatrics Ⅰ, the First Affiliated Hospital of Zhengzhou University and Department of Pediatrics, the Affiliated Hospital of Inner Mongolia Medical University from January 2013 to June 2022.The rheumatic patients complicated with PAH were followed up by telephone on June 30, 2022, and their clinical symptoms, treatment, follow-up, and prognosis data were collected.According to different treatment methods, the patients were divided into different clinical subgroups. The change of PAH was analyzed. The t-test was used for comparison between groups. P<0.05 was statistically significant. Results:A total of 24 cases were enrolled, with 7 males and 17 females.The average onset age of PAH was (10.97±3.79) years old.The median duration of PAH was 6.00 (32.20) months.The average pulmonary artery pressure was (51.71±17.66) mmHg(1 mmHg=0.133 kPa). There were 9 cases of systemic lupus erythematosus, 5 cases of Takayasu′s arteritis, 3 cases of juvenile dermatomyositis, 3 cases of undifferentiated connective tissue disease, 2 cases of systemic juvenile idiopathic arthritis, 1 case of Behcet′s disease, and 1 case of Kawasaki disease.Among 24 cases, the common symptoms were fever (14 cases), fatigue (10 cases) and dyspnea (7 cases). Of the 24 cases, 10 cases were complicated with hydropericardium, 9 cases with valve regurgitation, and 5 cases with decreased systolic and/or diastolic function.Lung changes were observed in 17 cases.Eleven cases were tested for B-type natriuretic peptide (BNP), and the BNP levels were all elevated in them (11 cases), with a median BNP of 3 073 (10 645) ng/L.After the first occurrence of PAH, 12 cases were treated with Methylprednisolone therapy, 10 cases received Cyclophosphamide therapy, and 2 cases who were both systemic lupus erythematosus, underwent blood purification.In the treatment of PAH, 11 cases were treated with pulmonary artery pressure reduction, and 7 of the 11 cases took PAH-targeted drugs.The mean decrease of the average pulmonary artery pressure in children receiving the targeted therapy[(44.80±24.08) mmHg] was significant higher than that in children not receiving the targeted therapy [(16.15±17.25) mmHg] ( t=2.661, P=0.016). Twenty children were reexamined and/or followed up, and the average course of PAH at the telephone follow-up was (36.29±26.67) months.The pulmonary arterial hypertension in 6 cases completely recovered, with median recovery time of 8.00 (13.47) months, but 2 of them died after the complete recovery.The pulmonary arterial hypertension improved in 11 children, 1 of whom died and the remaining children were in stable condition.The pulmonary arterial hypertension worsened in 2 children, 1 of them improved previously but aggravated recently, and the other child did not monitor pulmonary artery pressure and died during telephone follow-up. Conclusions:Rheumatic diseases complicated with PAH are rare and most often diagnosed in severe rheumatic children.It can lead to death, and is commonly accompanied by notably elevated BNP levels.The patients who have early PAH detection, intensive treatment of the primary disease, symptomatic and targeted pulmonary artery pressure reduction show a better prognosis.
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Objective:To summarize the clinical features of chronic non-bacterial osteomyelitis (CNO) in children.Methods:Clinical data of 8 CNO patients admitted to the Department of Rheumatology and Immunology, Children′s Hospital Affiliated to Capital Institute of Pediatrics from March 2014 to December 2020 were retrospectively analyzed.The clinical characteristics of 8 children with CNO were summarized and compared with those reported abroad.Results:A total of 8 CNO patients were recruited, involving 3 males and 5 females with the mean age of onset (7.2±3.2)years, and the average diagnosis time 25.9 months, respectively.The common clinical symptoms included bone pain (7 cases, 87.5%), arthritis (4 cases, 50.0%), and fever (3 cases, 37.5%). The main manifestations on X-ray and CT scans were bone destruction and progressive osteosclerosis.Magnetic resonance imaging (MRI) showed bone marrow edema, periostitis, soft tissue swelling, and enhancement.All of them had more than one site of bone involvement.Seven patients(87.5%) had bilateral bone involvement, with the most common site of tibia (22.0%), followed by femur (17.1%) and mandible (9.8%). Bone biopsy was performed in 8 patients, and 4 cases showed osteonecrosis, 4 cases showed bone fibrosis and 2 cases showed osteomyelitis.The etiological examination of the bone was negative.Eight children received non-steroid anti-inflammatory drugs alone or in combination with glucocorticoids, disease-modifying antirheumatic drugs (DMARDs), bisphosphonates or tumor necrosis factor-α(TNF-α) antagonists.After treatment, the patients were followed up for 3 months to 2 years.Eight children improved.Their inflammatory indexes were normal, and had no disability, teratology or multiple organ damage.Conclusions:Pediatric CNO is more common in children of school age, with a long course of disease.The main manifestations are multi-site bone pain and arthritis.Imaging studies indicate multiple bone involvement, which is more common at lower extremities.Non-steroids anti-inflammatory drugs, glucocorticoids, DMARDs, bisphosphonates and TNF-α antagonists are effective to CNO.
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Objective:To improve the understanding of Takayasu′s arteritis (TA) and its diagnosis and treatment by analyzing and summarizing the clinical characteristics of TA in children at different ages.Methods:Clinical and follow-up data of 41 children with TA admitted in Children′s Hospital, Capital Institute of Pediatrics between January 2010 and May 2020 were retrospectively analyzed.Based on the cut-off age of 3 years, children with TA were divided into older group and younger group.Clinical characteristics, involvement of the coronary artery, blood pressure control and growth restriction between 2 groups were analyzed.Counting data were expressed as percentage and case, and compared by the Chi- square test. Results:Among the 17 children with TA in younger group, there were 8 males and 9 females.There were 6 males and 18 females in older group.The general type was most common in younger group, with 10 cases (58.8%). In older group, thoracic and abdominal aortic type was the most common, with 13 cases (54.2%). The most common clinical manifestation in younger group was fever, with 13 cases (76.5%). In older group, 19 patients (79.2%) had hypertension.Lower hemoglobin (Hb) was detected in younger group.Leukocyte count, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) increased in all patients (100.0%). In older group, leukocyte count increased in 6 cases (25.0%), ESR increased in 13 cases (54.2%), and CRP increased in 11 cases (45.8%). The coronary artery and its branches (anterior descending branch and circumvolute branch) were the mostly affected in younger group, with 16 cases (94.1%). The subclavian artery was the most commonly involved in older group (15 cases, 62.5%). All TA children in younger group were in the active stage.Among them, 8 cases were treated with biological agents alone, 3 cases were treated with glucocorticoid alone, 5 cases were treated with the combination of glucocorticoid and biological agents, and 1 case was treated with glucocorticoid first, and then transferred to biological agents due to the poor effect.In older group, there were 18 active-stage patients (75.0%), and 2 refused treatment.Sixteen active patients and 6 inactive patients were treated with glucocorticoid, involving 19 cases treated with glucocorticoid combined with Cyclophosphamide, and 3 cases treated with glucocorticoid combined with biologics.There were 16 cases of coronary artery involvement in younger group and only 1 case in older group ( P<0.01). In younger group, 9 patients had growth restriction, while none was detected in older group ( P<0.01). The blood pressure of younger group was all controlled, which was not satisfactorily controlled in 16 cases of older age ( P<0.01). The incidence of general type and active stage in younger group was higher than that of older group without significant difference ( P>0.05). Conclusions:The clinical characteristics of TA vary at different ages.TA progresses more rapidly in younger children, which are more prone to the involvement of extensive vessels, the coronary arteries and other vessels, and the effects of drugs on growth and development should be well concerned.Older TA patients can be alleviated into the inactive phase by themselves, which is mainly characterized as the involvement of large vessels and hypertension sequelae.
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Objective:To observe the clinical efficacy of intra-articular injection with Triamcinolone acetonide on the treatment of juvenile idiopathic arthritis (JIA).Methods:The clinical data of 26 children diagnosed with JIA undergoing the intra-articular injection of Triamcinolone acetonide for the joints with obvious swelling and pain at the Children′s Hospital Affiliated to Capital Institute of Pediatrics from October 2018 to December 2019 who were retrospectively analyzed.Erythrocyte sedimentation rate (ESR) and C-reactive protein(CRP) were tested before and after the application of Triamcinolone acetonide.Detailed clinical manifestations were recorded.The nonparametric Kruskal- Wallis test was used to compare the differences in clinical evaluation indicators and changes in laboratory tests at diffe-rent treatment times. Results:Among the 26 children, 8 were boys and 18 were girls.After the intra-articular injection of Triamcinolone acetonide, 9 cases (34.62%) achieved complete remission, 15 cases(57.69%) achieved partial remission, and 2 cases (7.69%) were not responsive to the intra-articular injection.The overall therapeutic efficacy was 92.31%.Compared with pre-treatment period, from 4 weeks after treatment, assessment of disease activity by the physicians and parents of the children was significantly improved after 4-week treatment, and the number of active joints, ESR and CRP and the Juvenile Arthritis Disease Activity Score with 27 joints (JADAS 27) gradually decreased, and the differences were statistically significant (all P<0.05). No adverse drug reactions were seen during the treatment and follow-up period. Conclusions:Intra-articular injection of Triamcinolone acetonide is effective in contro-lling joint symptoms of JIA with less adverse events.
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Objective:To clarity the clinical features of juvenile dermatomyositis (JDM) with positive anti-melanoma differentiation associated gene 5 (MDA5) antibody.Methods:Retrospective study.Clinical data of 11 anti-MDA5 autoantibody-positive JDM patients in the Department of Rheumatology and Immunology, Children′s Hospital Affiliated to Capital Institute of Pediatrics from January 2016 to January 2019 were retrospectively recruited for analyzing their clinical characteristics, pulmonary imaging and pulmonary function, thus summarizing treatment experiences.Results:A total of 11 children with anti-MDA5 autoantibody-positive JDM were recruited, involving 2 males and 9 females, with the average onset age of 1-13 (5.8±4.2) years.Clinical manifestations included rash in 11 cases (100.0%), arthritis in 5 cases (45.5%), and myasthenia in 4 cases (36.4%). Muscle enzyme elevated in 10 cases (90.9%) and serum ferritin (SF) elevated in 9 patients (81.8%). Ten cases (90.9%) showed interstitial lung disease (ILD), manifesting as ground glass opacity at subpleural area on CT scans, restrictive ventilation and decreased diffusion function on lung function test, while respiratory symptoms were absent.All patients were treated with glucocorticoid combined with immunosuppressor.Case 2 developed into rapid progressive pulmonary interstitial disease (RPILD), and died of respiratory failure 2 months later.The remaining was followed up for 1-2 years, and the ILD was relieved.Conclusions:All recruited children with anti-MDA5 autoantibody-positive JDM presented typical rash, and mild muscle weakness with a greater tendency to arthritis.Chinese pediatric patients are prone to complicate with ILD with no respiratory symptoms, but ground glass opacity at subpleural area on CT, and restrictive ventilation and decreased diffusion function on lung function test can be detected.Elevated SF is associated with the development of ILD.Glucocorticoid combined with immunosuppressive therapy is effective to JDM with ILD, but ineffective for RPILD.The mortality of anti-MDA5 autoantibody-positive JDM is high without an effective treatment.
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Purpose@#The purpose of this study was to investigate the influences of sex comb on midleg like-2 (SCML2) on hepatocellular carcinoma (HCC) and potentially related mechanisms. @*Materials and Methods@#SCML2 expression in tumor tissues and cells was analyzed using the TCGA database and/or qRT-PCR. The proliferation of HCC cells was detected by CCK-8, colony formation, and EdU assays. The migration and invasion of HCC cells were detected by transwell and wound healing assays. Apoptosis of HCC cells was determined by flow cytometry. Additionally, qRT-PCR and Western blot were used to detect the expression of SCML2 and Wnt/β-catenin/epithelial–mesenchymal transition (EMT) signaling. A xenograft model in mice was established to verify the in vitro findings. @*Results@#We found that SCML2 was highly expressed in HCC tissues and cells and that high expression of SCML2 was correlated with poor prognosis in HCC patients. SCML2 overexpression promoted proliferation, invasion, and migration and repressed apoptosis of HCC cells. The reverse results were obtained in SCML2-silenced cells. Further, we found that SCML2 activated the Wnt/β-catenin/EMT pathway. SCML2 silencing reduced the protein levels of Wnt3a, β-catenin, N-cadherin, Vimentin, and Snail and enhanced E-cadherin protein expression both in vivo and in vitro. @*Conclusion@#SCML2 silencing inhibits the proliferation, migration, and invasion of HCC cells by regulating the Wnt/β-catenin/ EMT pathway.
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Objective:To investigate the clinical features, diagnosis and treatment of systemic juvenile idiopathic arthritis (SJIA) complicated with macrophage activation syndrome (MAS).Methods:From January 1st, 2018 to January 1st, 2020, 7 cases of SJIA-MAS were diagnosed. Their clinical and laboratory data were collected and summarized.Results:In these 7 cases, 2 were males and 5 were females, the ratio of male to female was 2∶5. The age range was 11 months to 2 years old. The course of disease was 14 to 32 days. The clinical manifestations included fever and rash in 7 without arthritis; hepatomegaly, splenomegaly and lymphadenopathy in 7; hematological involvement in 7; nervous system involvement in 2; digestive system involvement in 7; respiratory system involvement in 7; cardiovascular involvement in 3. White blood cell was decreased in 1 case, platelet was decreased in 1 case and hemoglobin was decreased in 7 cases. Ferritin, triglyceride, alanine transaminas and aspartate aminotransferase were increased in 7 cases, fibrinogen was significantly decreased in 7 cases, and direct bilirubin was increased in 4 cases. IL-2R was significantly increased. Hemophagocytosis was observed in bone marrow of 4 cases. Cerebrospinal fluid protein was 2 005 mg/L in 1 case. All the 7 cases were tested for exon genes, and no pathogenic mutation was found. All of the 7 cases showed lung lesions in chest CT scan. Multiple demyelinating lesions were found in 1 case by head magnetic resonance imaging. One case was treated with high-dose intravenous methylprednisolone combined with IL-6 receptor antagonist(tocilizumab). The other 6 cases were treated with high-dose intravenous methylprednisolone combined with cyclosporine A (CsA). Two cases were treated with Janus kinases inhibitor(tofacitinib). After treatment, 7 cases got relieved, no death, no recurrence oocurred during the follow-up.Conclusion:Acute onset, multiple organ involvement and no joint inflammation are prominent in MAS of infants and toddlers. High fever, proressive reduction of blood cells and increase of SF are significant in SJIA-MAS. High dose glucocorticoid combined with CsA can benefit in most cases, and some severe cases need to be treated with biological agents.
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Objective:To evaluate the value of serum cytokine level in the efficacy of tocilizumab for the treatment of systemic-onset juvenile idiopathic arthritis.Methods:30 cases with SoJIA hospitalized in Capital Institute of Paediatrics from June 2016 to October 2018 were treated with Interleukin-6 receptor antagonist(tocilizumab) injection. Among them, 20 were males(67%) and 10 were females(33%). The age at diagnosis was between 0.84 to 13years. Whiteblood cell, C-reactive protein, erythrocyte sedimentation rate, serum interleukin(IL-6, IL-2R, IL-8, IL-10, IL-1β) and tumor necrosis factor-alpha levels were observed before treatment, after the 2nd week, after the 6th week and after the 22nd week.Mann-Whitney nonparametric test and Chi-square test were used to analyze the data of cytokines pre and after-treatment.Results:All of the 30 cases had fever before medication. The fever disappeared in 28 cases after using tocilizumab. One case stopped using tocilizumab because of allergic reaction and one case stopped because of poor efficacy. Among 28 cases with normal body temperature after medication, the arthritis and rash manifestations were significantly improved. WBC, AESR and CRP were all lower than those before medication. Within these 28 cases, the serum IL-6 level was168.50(67.40-589.25) pg/mL pre-treatment, 107.50(28.03-281.50) pg/mL after the 2nd week. There was no statistical difference between them( Z=-1.754, P>0.05). The serum IL-6 level was 64.05 (19.90-130.75) pg/mL after the 6th week and 24.80 (3.45-95.40) pg/mL after the 22nd week. Compared with pre-treatment, they were all lower than pre-treatment levels( Z=-2.942,-3.334, P<0.01,<0.01).Serum IL-2R level was 740.50(510.00-1 161.00)U/mL after the 2nd week, 796.50 (534.00-1 008.00) U/mL after the 6th week and 688.00 (527.00-889.50) U/mL after the 22nd week. Compared with pre-treatment [1 322.50(812.00-1 659.00)U/mL], they were all lower than pre-treatment levels ( Z=-2.818,-3.130,-3.466, P<0.01, <0.01, <0.01). Serum TNF-alpha level was 23.70 (20.30-41.23) pg/ml after the 2nd week, 26.75(16.83-47.03) pg/ml after the 6th week,18.60(13.10-34.90) pg/ml after the 22nd week. Compared with pre-treatment [26.50(20.55-37.43) pg/ml], there were no statistical difference between after and pre-treatment( Z=0,-0.560,-1.954, P>0.05,>0.05,>0.05). Serum IL-8 level was 200.85(95.43-364.00)pg/ml after the 2nd week, 194.50(50.75-433.00)pg/ml after the 6th week, 161.50 (38.98-308.00)pg/ml after the 22nd week. Compared with pre-treatment [96.20(59.75-371.75) pg/ml], there were no statistical difference between after and pre-treatment( Z=-0.86,-0.131,-0.186, P>0.05,>0.05,>0.05). There was no statistical difference between after the 2nd week and pre-treatment in the IL-10 level(χ 2=2.33, P>0.05). The IL-10 level after 6th week and after 22nd week were all lower than pre-treatment levels(χ 2=4.08, 4.08, P<0.05, <0.05). There were no statistical difference between after and pre-treatment(χ 2=0.084, 2.504,3.818, P>0.05,>0.05,>0.05)in IL-1β level. Conclusion:After treatment with tocilizumab, the levels of serum IL-6 and IL-2R are helpful to assess the activity of SoJIA and the efficacy of therapy.
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Objective@#To evaluate the value of serum cytokine level in the efficacy of tocilizumab for the treatment of systemic-onset juvenile idiopathic arthritis.@*Methods@#30 cases with SoJIA hospitalized in Capital Institute of Paediatrics from June 2016 to October 2018 were treated with Interleukin-6 receptor antagonist(tocilizumab) injection. Among them, 20 were males(67%) and 10 were females(33%). The age at diagnosis was between 0.84 to 13years. Whiteblood cell, C-reactive protein, erythrocyte sedimentation rate, serum interleukin(IL-6, IL-2R, IL-8, IL-10, IL-1β) and tumor necrosis factor-alpha levels were observed before treatment, after the 2nd week, after the 6th week and after the 22nd week.Mann-Whitney nonparametric test and Chi-square test were used to analyze the data of cytokines pre and after-treatment.@*Results@#All of the 30 cases had fever before medication. The fever disappeared in 28 cases after using tocilizumab. One case stopped using tocilizumab because of allergic reaction and one case stopped because of poor efficacy. Among 28 cases with normal body temperature after medication, the arthritis and rash manifestations were significantly improved. WBC, AESR and CRP were all lower than those before medication. Within these 28 cases, the serum IL-6 level was168.50(67.40-589.25) pg/mL pre-treatment, 107.50(28.03-281.50) pg/mL after the 2nd week. There was no statistical difference between them(Z=-1.754, P>0.05). The serum IL-6 level was 64.05 (19.90-130.75) pg/mL after the 6th week and 24.80 (3.45-95.40) pg/mL after the 22nd week. Compared with pre-treatment, they were all lower than pre-treatment levels(Z=-2.942,-3.334,P<0.01,<0.01).Serum IL-2R level was 740.50(510.00-1 161.00)U/mL after the 2nd week, 796.50 (534.00-1 008.00) U/mL after the 6th week and 688.00 (527.00-889.50) U/mL after the 22nd week. Compared with pre-treatment [1 322.50(812.00-1 659.00)U/mL], they were all lower than pre-treatment levels (Z=-2.818,-3.130,-3.466, P<0.01, <0.01, <0.01). Serum TNF-alpha level was 23.70 (20.30-41.23) pg/ml after the 2nd week, 26.75(16.83-47.03) pg/ml after the 6th week,18.60(13.10-34.90) pg/ml after the 22nd week. Compared with pre-treatment [26.50(20.55-37.43) pg/ml], there were no statistical difference between after and pre-treatment(Z=0,-0.560,-1.954,P>0.05,>0.05,>0.05). Serum IL-8 level was 200.85(95.43-364.00)pg/ml after the 2nd week, 194.50(50.75-433.00)pg/ml after the 6th week, 161.50 (38.98-308.00)pg/ml after the 22nd week. Compared with pre-treatment [96.20(59.75-371.75) pg/ml], there were no statistical difference between after and pre-treatment(Z=-0.86,-0.131,-0.186,P>0.05,>0.05,>0.05). There was no statistical difference between after the 2nd week and pre-treatment in the IL-10 level(χ2=2.33, P>0.05). The IL-10 level after 6th week and after 22nd week were all lower than pre-treatment levels(χ2=4.08, 4.08, P<0.05, <0.05). There were no statistical difference between after and pre-treatment(χ2=0.084, 2.504,3.818,P>0.05,>0.05,>0.05)in IL-1β level.@*Conclusion@#After treatment with tocilizumab, the levels of serum IL-6 and IL-2R are helpful to assess the activity of SoJIA and the efficacy of therapy.
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Objective@#To improve the understanding and diagnosis and treatment level of infant with Takayasu arteritis (TA) by analyzing the clinical features of 14 pediatric patients and reviewing related articles.@*Methods@#The clinical and follow-up data of infants with TA who were admitted to the Children′s Hospital Affiliated to Capital Institute of Pediatrics between July 2016 and May 2019 were retrospectively analyzed.By reviewing related articles, the clinical features of this disease were summarized.@*Results@#The age of 14 patients (including 6 males and 8 females) were between 1 month and 23 days and 28 months.The most common clinical manifestations were fever in 10 cases (71.4%), hypertension in 9 cases (64.3%), weak or no pulse in 5 cases (35.7%). According to the clinical type of lesion vessels, 11 cases (78.5%) were generalized type, 3 cases (21.4%) were brachiocephalic artery type, and there was no thoracic abdominal aorta or single pulmonary artery type in this group.Among 14 infants with TA, 12 cases had common carotid artery, carotid artery, subclavian artery, coronary artery and its branches (anterior descending branch, circumflex branch) involved (85.7%); 11 cases had renal artery involved (78.6%); 9 cases had radial artery involved (64.2%); 8 cases had abdominal aorta involved (57.1%); 6 cases had descending aorta involved (42.9%); 6 cases had thoracic aorta involved (42.9%); 6 cases had superior mesenteric artery involved (42.9%); 5 cases had femoral artery involved (35.7%); 5 cases had pulmonary artery involved (35.7%); and 4 cases had brachial artery involved (28.6%). In those 14 patients, 11 cases were misdiagnosed, and 3 cases had unclear diagnosis, with misdiagnosis duration of 18 days to 2 months.In misdiagnosed cases, 8 cases were misdiagnosed as atypical Kawasaki disease.Among those 14 cases, the ranges of most lesions were gradually decreased, and the slightly involved vessels even completely returned to normal state after treatment in 7 cases.The vascular imaging showed no significant exacerbation or improvement in 4 cases.Nine cases developed hypertension, the blood pressure of whom could be controlled within normal range with hypotensive drugs which could not be interrupted.Physical examination found weak or no pulse in 5 cases who were not improved.Among 14 patients, 7 cases showed normal development, while the height and body mass of another 7 cases were the 25th percentile below those of normal children of the same age.All 14 patients were followed up for 2-22 months and received regular treatment without recurrence.@*Conclusions@#TA patients aged less than 3 years tend to have more blood vessels involved, be in serious condition and have higher rate of misdiagnosis.The disease can be controlled quickly after treatment, but vascular diseases may be developed easily.Some patients have a poor prognosis.
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Objective To improve the understanding and diagnosis and treatment level of infant with Takayasu arteritis (TA) by analyzing the clinical features of 14 pediatric patients and reviewing related articles.Methods The clinical and follow-up data of infants with TA who were admitted to the Children's Hospital Affiliated to Capital Institute of Pediatrics between July 2016 and May 2019 were retrospectively analyzed.By reviewing related articles,the clinical features of this disease were summarized.Results The age of 14 patients (including 6 males and 8 females) were between 1 month and 23 days and 28 months.The most common clinical manifestations were fever in 10 cases (71.4％),hypertension in 9 cases (64.3％),weak or no pulse in 5 cases (35.7％).According to the clinical type of lesion vessels,11 cases (78.5％) were generalized type,3 cases (21.4％) were brachiocephalic artery type,and there was no thoracic abdominal aorta or single pulmonary artery type in this group.Among 14 infants with TA,12 cases had common carotid artery,carotid artery,subclavian artery,coronary artery and its branches (anterior descending branch,circumflex branch) involved (85.7％);11 cases had renal artery involved (78.6％);9 cases had radial artery involved (64.2％);8 cases had abdominal aorta involved (57.1％);6 cases had descending aorta involved (42.9％);6 cases had thoracic aorta involved (42.9％);6 cases had superior mesenteric artery involved (42.9％);5 cases had femoral artery involved (35.7％);5 cases had pulmonary artery involved (35.7％);and 4 cases had brachial artery involved (28.6％).In those 14 patients,11 cases were misdiagnosed,and 3 cases had unclear diagnosis,with misdiagnosis duration of 18 days to 2 months.In misdiagnosed cases,8 cases were misdiagnosed as atypical Kawasaki disease.Among those 14 cases,the ranges of most lesions were gradually decreased,and the slightly involved vessels even completely returned to normal state after treatment in 7 cases.The vascular imaging showed no significant exacerbation or imnprovement in 4 cases.Nine cases developed hypertension,the blood pressure of whom could be controlled within normal range with hypotensive drugs which could not be interrupted.Physical examination found weak or no pulse in 5 cases who were not improved.Among 14 patients,7 cases showed normal development,while the height and body mass of another 7 cases were the 25th percentile below those of normal children of the same age.All 14 patients were followed up for 2-22 months and received regular treatment without recurrence.Conclusions TA patients aged less than 3 years tend to have more blood vessels involved,be in serious condition and have higher rate of misdiagnosis.The disease can be controlled quickly after treatment,but vascular diseases may be developed easily.Some patients have a poor prognosis.
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Objective To investigate the early typing diagnostic and predictive value of anti-keratin antibodies(AKA), anti-perinuclear factor(APF) and anti-citrullinated protein antibodies(ACPA) in patients of juvenile idiopathic arthritis (JIA). Methods A retrospective study was conducted to collect 144 cases of JIA who were hospitalized in Capital Institute of Pediatrics from December 2013 to June 2016 and followed up for at least one year.Among them,66 were males (46％) and 78 were females (54％).The age at diagnosis was between 1 year 5 months to 15 years 9 months.144 patients were tested for AKA,ACPA,APF and TNFα upon admission. Chi-square test or Fisher exact test were used to compare the positive rates of three antibodies among different subtypes. Mann-Whitney nonparametric test and Chi-square test or Fisher exact test were used to analyze the data of prognosis between antibody-positive group and antibody-negative group in the course of disease. Results In 144 patients, 49(34％) were classified as systemic arthritis, 28 (19.4％) as polyarthritis, 61(42.3％) as oligoarthritis, and 6(4.2％) as enthesitis-associated arthritis. 52 cases (36.1％) were positive for one antibody or more antibodies of AKA/APF/ACPA at the early stage,14(9.7％) were AKA positive, 44(30.6％) were ACPA positive and 12(8.3％) were APF positive. The positive rates of ACPA/AKA/APF antibodies were significantly different among different subtypes(χ2=33.863,26.860,14.395;P<0.01,<0.01,<0.05).The rates in polyarthritis were higher than those in systemic arthritis and oligoarthritis;In 95 children with non-systemic form,the level of TNFαin antibody-positive group(43 cases)was higher than that in antibody-negative group(52 cases)at the early stage(Z=4.785, P<0.01);144 patients were followed up for at least one year,the rates of patients who accepted biologic therapies were significantly different between antibody-positive group and antibody-negative group (50％ vs 25％). So do the rates of patients with joint deformities(17.3％vs 2.2％)and with important joints involvement (hip and axis joints)(59.6％vs 14.1％)(χ2=9.249, 10.875, 32.392; P<0.01, <0.01, <0.01). Further more, the number of joints involved in the antibody-positive group (7.07 ± 3.85) was significantly more than that in the antibody-negative group (2.31 ± 1.64)(F=63.822,P<0.01). Conclusions AKA,APF and ACPA are important in the early typing diagnosis of JIA,and may be closely related to the prognosis of patients with JIA.
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Objective@#To investigate the early typing diagnostic and predictive value of anti-keratin antibodies(AKA), anti-perinuclear factor(APF) and anti-citrullinated protein antibodies(ACPA) in patients of juvenile idiopathic arthritis (JIA).@*Methods@#A retrospective study was conducted to collect 144 cases of JIA who were hospitalized in Capital Institute of Pediatrics from December 2013 to June 2016 and followed up for at least one year.Among them,66 were males (46%) and 78 were females (54%).The age at diagnosis was between 1 year 5 months to 15 years 9 months.144 patients were tested for AKA,ACPA,APF and TNFα upon admission. Chi-square test or Fisher exact test were used to compare the positive rates of three antibodies among different subtypes. Mann-Whitney nonparametric test and Chi-square test or Fisher exact test were used to analyze the data of prognosis between antibody-positive group and antibody-negative group in the course of disease.@*Results@#In 144 patients, 49(34%) were classified as systemic arthritis, 28 (19.4%) as polyarthritis, 61(42.3%) as oligoarthritis, and 6(4.2%) as enthesitis-associated arthritis. 52 cases (36.1%) were positive for one antibody or more antibodies of AKA/APF/ACPA at the early stage, 14(9.7%) were AKA positive, 44(30.6%) were ACPA positive and 12(8.3%) were APF positive. The positive rates of ACPA/AKA/APF antibodies were significantly different among different subtypes(χ2=33.863,26.860,14.395; P<0.01,<0.01,<0.05).The rates in polyarthritis were higher than those in systemic arthritis and oligoarthritis; In 95 children with non-systemic form, the level of TNFα in antibody-positive group (43 cases) was higher than that in antibody-negative group (52 cases) at the early stage(Z=4.785, P<0.01);144 patients were followed up for at least one year,the rates of patients who accepted biologic therapies were significantly different between antibody-positive group and antibody-negative group (50% vs 25%). So do the rates of patients with joint deformities (17.3% vs 2.2%) and with important joints involvement (hip and axis joints) (59.6% vs 14.1%) (χ2=9.249,10.875,32.392; P<0.01,<0.01,<0.01). Further more, the number of joints involved in the antibody-positive group (7.07±3.85) was significantly more than that in the antibody-negative group (2.31±1.64) (F=63.822, P<0.01).@*Conclusions@#AKA,APF and ACPA are important in the early typing diagnosis of JIA,and may be closely related to the prognosis of patients with JIA.
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Objective To summarize the clinical characteristics of Beh?et's disease (BD) in children with gastrointestinal involvement. Methods We retrospectively analyze the children BD with gastrointestinal involvement who were diagnosed in our hospital in recent 10 years. Results Twenty-two children were identified. The average age of onset was(6.1±4.0) years. The time from disease onset to clinical diagnosis was (1.2±2.1) years on average. Fifteen children had abdominal pain, diarrhea and hematochezia. Seven cases had positive endoscopic findings without any gastrointestinal symptoms. Twenty cases received corticosteroids therapy, 13 cases of them were treated with Cyclophosphamide/Methotrexate (CTX/MTX), 3 refractory cases were treated with biologics. Patients were followed up for (28±32) month on average. Eight patients' condition was stable, 7 patients were refractory, 3 patients died, 4 patients were lost to follow-up. At the same term, 5 patients without gastrointestinal involvement who received corticosteroids and CTX/MTX therapy were stable. Conclusion It is difficult to diagnose children BD at early stage. Gastrointestinal involvement may not be found, while the gastrointestinal endoscopy is of great importance in the diagnosis of the disease. Gluco-corticoid combined with immunosuppressive agents are effective. As to refractory patients, biological agent might be used although the recurrence is common. Compared with BD without gastrointestinal involvement, children BD with gastrointestinal involvement have serious condition and poor prognosis.
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Objective@#To analyze the clinical characteristics of eperythrozoonosis complicated with hemophagocytic syndrome (HPS) in 4 children.@*Methods@#Four patients diagnosed with eperythrozoonosis complicated with HPS in the Children's Hospital Affiliated Capital Institute of Pediatrics during the period from June 2014 to July 2016 were enrolled. The clinical manifestations, laboratory examination data and therapeutic strategies were analyzed. A literature search (search terms included 'eperythrozoonosis’ and 'hemophagocytic syndrome’) was conducted using CNKI, Wanfang database, Chinese biomedical literature database and PubMed to include recently published studies (searched from the database establishment to January 2017).@*Results@#Four patients were included in the study. One was boy and the other three were girls. The age range of the 4 patients was between 9 months and 17 years (9 months, 2 years and 17 years, 11 months respectively). All the patients presented with recurrent high fever. During the course of fever, 3 patients presented with rash, and 2 patients presented with joint pain and swelling, which mimicked systemic juvenile idiopathic arthritis. Only 1 patient had the contact history of infectious disease. All patients had normal or decreased white blood cell count ((0.80-13.12)×109/L), suffered from varied degrees of anemia and showed the increased C reactive protein (13.0-84.7 mg/L) anderythrocyte sedimentation rate (13-72 mm/1 h). Examination of peripheral blood smears confirmed eperythrozoonosis. After fever continued about 1 month, all the 4 patients rapidly progressed. Among the 4 patients, 1 patient died for giving up further therapy, and the other 3 patients completely recovered after treatment, including azithromycin for the treatment of eperythrozoonosis, and high-dose intravenous methylprednisolone pulse therapy and human immunoglobulin for the treatment of HPS. For the disease not satisfactory, the hemophagocytic lymphohistiocytosis-2004 (HLH-2004) protocol is given. After the hospitalization of 1 to 2 months, the conditions improved and the children were discharged from hospital. Three patients were followed up for 8 months to 2 years, and their conditions were stable. In the PubMed database, no report was found. Nine cases of children with eperythrozoonosis were found in CNKI, Wanfang database and Chinese biomedical literature database, and 1 case was complicated with HPS. These findings, taken together our report, provided the data of 5 children with eperythrozoonosis complicated with HPS (4 cases were younger than 2 years old). A patient had contact history of infectious disease. Five patientss showed fever of unknown origin. All the patients had severe eperythrozoonosis, and 2 cases at younger age died.@*Conclusions@#Children with eperythrozoonosis often present with the protracted fever of unknown origin, and clinical manifestations mimic those of juvenile idiopathic arthritis (systemic type). The patients with eperythrozoonosis of mild-to-moderate disease severity may have a good prognosis. Children with severe eperythrozoonosis, especially those HPS cases with early onset before 2 years old, may have high risk of mortality. Once the patient's condition aggravates in the course of fever, HPS should be highly suspected. For the patients with eperythrozoonosis complicated with HPS, early diagnosis and the combination of anti-infection with the treatment of HPS are crucial for a good prognosis. For the treatment of HPS, HLH-2004 protocol is recommended.
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Objective@#To summarize the clinical data of 15 patients with fibrodysplasia ossificans progressiva (FOP), follow up and analyze the characteristics of the joint involvement in FOP.@*Method@#From May 2005 to December 2016, fifteen FOP cases had been diagnosed in the Children′s Hospital Capital Institute of Pediatrics. All medical records and follow-up data were collected and a retrospective analysis was made on the joint involvement in FOP. Pearson correlation analysis was used for data, P<0.05 for the difference was statistically significant.@*Result@#There were 8 males and 7 females in 15 cases. The age of onset was 2(1-6)years. The age at diagnosis was 6 (4-9) years. All cases had hallux valgus deformity and bone mass formation. Twelve cases had joints involvement on enrollment into this study: 8 cervical vertebra, 7 shoulder joint, 5 hip joint, 4 elbow joint, 3 wrist joint, 2 temporomandibular joint, 2 lumbar vertebra. The age of diagnosis and duration of disease were positively correlated with the number of the involved joints (r=0.523, 0.628; P=0.045, 0.012); mild changes were found in joint imaging. Thirteen cases received telephone follow-up, the average duration of follow-up was 6(3-7)years, no change in 11 cases, disease progress in 2 cases.@*Conclusion@#Joint involvement is a common complication of FOP, especially the cervical vertebra.Multiple joints involvement, dominant functional impairment, and mild imaging changes are the characteristics of joint lesions caused by FOP.The number of involved joints gradually increases with increase of age of the patients and the prolonged course of the disease.
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Objective@#To analyze the clinical characteristics and infection of children with Takayasu′s arteritis(TA) for improving the awareness of the disease.@*Methods@#A retrospective study was performed on the 24 children with TA in our hospital.@*Results@#The average onset age was 9.3±3.2 years old, the ratio of male to female was 1∶3. The most common TA type was thoracic abdominal aortic type (54.2%) in clinical classification. The initial symptoms included high blood pressure, dizziness/headache, fever and fatigue, etc.Six cases (25%) had tuberculosis infection, including 1 case of tuberculosis. There are 3 patients (8.3%) with elevated O levels, 2 patients (8.3%) with EB virus infection and 1 patient (4.2%) with small viral B19 infection.@*Conclusions@#The onset of TA in children is complicated. It is important to take examinations carefully for early diagnosis, avoiding delay treatment and bad prognosis.