Objective:To analyze the clinical characteristics, treatment, response to treatment, prognosis, and the importance of early recognition and treatment of pediatric systemic lupus erythematosus (SLE) complicated with thrombotic microangiopathy (TMA).Methods:A retrospective summary of the clinical data of 5 children diagnosed with SLE complicated by TMA at Beijing Children′s Hospital, Capital Medical University, from November 2024 to January 2025.Results:Among the 5 children (1 boy and 4 girls, male-to-female ratio of 1∶4), the age of onset ranged from 11 years and 9 months to 14 years and 9 months. All cases had acute onset, rapid disease progression, severe illness, and involvement of multiple organs and systems. The disease activity of SLE was moderately to severely active when TMA was diagnosed. During the course of TMA, all 5 children exhibited varying degrees of hemolytic anemia, thrombocytopenia, renal dysfunction, and proteinuria. Elevated sC5b-9 levels were observed in all 5 children, with 3 showing severely reduced ADAMTS13 activity and 2 with elevated ADAMTS13 inhibitors. Two children had elevated sC5b-9, severely reduced ADAMTS13 activity, and elevated ADAMTS13 inhibitors simultaneously. Four children had newly diagnosed SLE with TMA and achieved stable condition within 2-3 weeks after aggressive treatment, including methylprednisolone pulse therapy, immunosuppressive agents, biologics, plasma infusion, plasma exchange, or dialysis. One child, who had been diagnosed with SLE for 8 years and had irregular oral medication for half a year, suddenly developed TMA with refractory hemolytic anemia and severe thrombocytopenia. This child responded poorly to methylprednisolone pulse therapy, cyclosporine, and eculizumab but showed improvement with plasma exchange. However, the condition was prone to relapse when the interval between plasma exchanges was prolonged. The child eventually responded well to low-dose rituximab and was discharged after 6 weeks of combined treatment. Four children were diagnosed with TMA within 1 week of admission and achieved stable condition after 3 months of follow-up, with no anemia or thrombocytopenia, negative proteinuria, normal complement C3 and C4 levels, and an SLE disease activity score of 0. One child, who had been treated with high-dose glucocorticoid, multiple immunosuppressive agents, and biologics at another hospital, was transferred and diagnosed with TMA 2 weeks after admission. The condition gradually stabilized after treatment with methylprednisolone pulse therapy, immunosuppressive agents, biologics, and plasma exchange. However, at 3-month follow-up, the child still had alopecia and proteinuria, with an SLE disease activity score of 6, indicating mild disease activity.Conclusion:For children with moderate to severe active SLE, especially those with acute onset, rapid disease progression, and prominent manifestations of anemia, thrombocytopenia, renal dysfunction, and poor response to high-dose methylprednisolone pulse therapy, the risk of TMA should be vigilantly monitored. Early diagnosis and aggressive treatment are crucial.

Objective:To analyze the clinical characteristics, treatment, response to treatment, prognosis, and the importance of early recognition and treatment of pediatric systemic lupus erythematosus (SLE) complicated with thrombotic microangiopathy (TMA).Methods:A retrospective summary of the clinical data of 5 children diagnosed with SLE complicated by TMA at Beijing Children′s Hospital, Capital Medical University, from November 2024 to January 2025.Results:Among the 5 children (1 boy and 4 girls, male-to-female ratio of 1∶4), the age of onset ranged from 11 years and 9 months to 14 years and 9 months. All cases had acute onset, rapid disease progression, severe illness, and involvement of multiple organs and systems. The disease activity of SLE was moderately to severely active when TMA was diagnosed. During the course of TMA, all 5 children exhibited varying degrees of hemolytic anemia, thrombocytopenia, renal dysfunction, and proteinuria. Elevated sC5b-9 levels were observed in all 5 children, with 3 showing severely reduced ADAMTS13 activity and 2 with elevated ADAMTS13 inhibitors. Two children had elevated sC5b-9, severely reduced ADAMTS13 activity, and elevated ADAMTS13 inhibitors simultaneously. Four children had newly diagnosed SLE with TMA and achieved stable condition within 2-3 weeks after aggressive treatment, including methylprednisolone pulse therapy, immunosuppressive agents, biologics, plasma infusion, plasma exchange, or dialysis. One child, who had been diagnosed with SLE for 8 years and had irregular oral medication for half a year, suddenly developed TMA with refractory hemolytic anemia and severe thrombocytopenia. This child responded poorly to methylprednisolone pulse therapy, cyclosporine, and eculizumab but showed improvement with plasma exchange. However, the condition was prone to relapse when the interval between plasma exchanges was prolonged. The child eventually responded well to low-dose rituximab and was discharged after 6 weeks of combined treatment. Four children were diagnosed with TMA within 1 week of admission and achieved stable condition after 3 months of follow-up, with no anemia or thrombocytopenia, negative proteinuria, normal complement C3 and C4 levels, and an SLE disease activity score of 0. One child, who had been treated with high-dose glucocorticoid, multiple immunosuppressive agents, and biologics at another hospital, was transferred and diagnosed with TMA 2 weeks after admission. The condition gradually stabilized after treatment with methylprednisolone pulse therapy, immunosuppressive agents, biologics, and plasma exchange. However, at 3-month follow-up, the child still had alopecia and proteinuria, with an SLE disease activity score of 6, indicating mild disease activity.Conclusion:For children with moderate to severe active SLE, especially those with acute onset, rapid disease progression, and prominent manifestations of anemia, thrombocytopenia, renal dysfunction, and poor response to high-dose methylprednisolone pulse therapy, the risk of TMA should be vigilantly monitored. Early diagnosis and aggressive treatment are crucial.

It is difficult to accurately distinguish pharyngitis caused by Group A Streptococcus(GAS) from other pathogens according to the clinical presentation alone, which cannot effectively guide the rational use of antimicrobials.The pharyngeal swab culture, rapid antigen detection test, nucleic acid test, and blood test can help definitively diagnose GAS pharyngitis.However, there are differences in different guidelines on who the laboratory test methods are intended for, interpretation of laboratory test results and so on.This article summarizes and analyses the laboratory diagnostic modalities and their characteristics, as well as recommendations for GAS pharyngitis in different guidelines to provide references for the clinical diagnosis, antimicrobial treatment, and further study of GAS pharyngitis.

Objective:To analyze the clinical characteristics of children with systemic lupus erythematosus (SLE) combined with thrombotic microangiopathy (TMA), and clarify the clinical outcomes and related risk factors of pediatric patients through their treatment and follow-up.Methods:This was a single-center retrospective case-control study. Children diagnosed with SLE combined with TMA between January 2017 and January 2023 at Beijing Children′s Hospital, Capital Medical University, were selected as the TMA group, and SLE children without TMA were selected as the control group.According to the prognosis, children in the TMA group were further divided into the good prognosis group and the poor prognosis group.The data of the children were collected, including age, gender, SLE disease activity, clinical presentations at the time of diagnosis and at the time of thrombosis, laboratory examinations, treatment strategies, prognosis, and follow-up results.The chi-square test and Z-test were used for comparison of count data.The t-test was used for comparison of metrological pairing data.The Fisher′s exact test was used to compare the differences between the 2 groups in categorical variables.The univariate Logistic regression was used to analyze the risk factors of poor prognosis. Results:There were 29 cases in the TMA group, and the incidence of TMA accounted for 2.53% of SLE patients; 33 cases were in the control group.The age at diagnosis of TMA was 13 years and 5 months (ranging from 9 years, 1 month and 5 days to 17 years and 4 months).The common clinical manifestations in order of prevalence were renal involvement (28 cases, 96.55%), hematologic involvement (26 cases, 89.66%), serous effusion (17 cases, 58.62%), rash (13 cases, 44.82%), and neurologic involvement (12 cases, 41.38%).Pleurisy or pericarditis, renal involvement and neurological involvement occurred more often in the TMA group than in the control group (17 cases vs.3 cases, 28 cases vs.10 cases, 12 cases vs.3 cases), and the TMA group showed less facial rash and arthritis than the control group (13 cases vs.25 cases, 4 cases vs.17 cases), and the differences were statistically significant (all P<0.05).The Systemic Lupus Erythematosus Disease Activity Index score in the TMA group [(24.14±9.42) scores] was significantly higher than that in the control group [(10.18±9.42) scores], and the difference was statistically significant ( t=3.233, P<0.05).The hemoglobin level, platelet count, and complement C3 level of the children in the TMA group were significantly lower than those in the control group, whereas the double stranded DNA antibody, lactate dehydrogenase, D-dimer, urea, creatinine, ferritin level, and urine protein quantitation were significantly higher than those in the control group, and the differences were statistically significant (all P<0.05).In the TMA group, 5 cases had decreased ADAMTS13 activity, and 5 cases had significantly increased complement C5b9.A total of 15 cases (51.72%) in the TMA group underwent renal biopsy, and 13 of them had combined renal TMA.In the TMA group, 28 patients (96.6%) received hormone therapy, 17 patients received plasma exchange, and 12 patients were treated with immunosuppressants and biologics; 19 patients (65.5%) improved, and 10 patients (34.5%) gave up the treatment due to deterioration of the disease.The urea level and peripheral blood fragmented erythrocyte rate in the good prognosis group were significantly lower than those in the poor prognosis group [(13.18±4.39) mmol/L vs.(21.16±10.14) mmol/L, t=2.975, P=0.006; 8/17 (47.06%) vs.7/7 (100%), χ2=5.929, P=0.015].The univariate Logistic regression analysis showed that the fragmented erythrocyte, ADAMTS13 activity and urea were the independent risk factors for poor prognosis (all P<0.05). Conclusions:SLE patients with moderate-to-severe disease activity, especially children with hemolytic anemia, thrombocytopenia, and renal dysfunction as prominent manifestations, should be alert to the risk of TMA.Early diagnosis and treatment are crucial.

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

The disease surveillance found that non-pharmaceutical interventions (NPIs) to prevent and control corona virus disease 2019 (COVID-19) also significantly affected the epidemiology of other infectious diseases.With the withdrawal of NPIs, the World Health Organization and other organizations have notified outbreaks of different infectious diseases.Compared with the epidemiology of these diseases before the COVID-19 pandemic, the outbreaks presented a number of atypical characteristics.The health workers should know and beware of these unusual changes to adjust strategies for diagnosis and treatment in time for the people′s health.

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

Group A Streptococcus (GAS) is a very important pathogen, especially for children.On a global scale, GAS is an important cause of morbidity and mortality.But the burden of disease caused by GAS is still unknown in China and also has not obtained enough attention.For this purpose, the expert consensus is comprehensively described in diagnosis, treatment and prevention of GAS diseases in children, covering related aspects of pneumology, infectiology, immunology, microbiology, cardiology, nephrology, critical care medicine and preventive medicine.Accordingly, the consensus document was intended to improve management strategies of GAS disease in Chinese children.

  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.

A 6-year-old girl presented with recurrent skin rash at the initial stage, recent joint pain, and neutrophilia was found during a routine blood test. After a multidisciplinary case discussion, she was diagnosed with chronic neutrophil leukemia, and the symptoms were relieved after hydroxyurea and luxolitinib treatment. She received the allogeneic hematopoietic stem cell transplantation subsequently. At present, she is in stable condition and under follow-up. Chronic neutrophil leukemia is a rare disease, which rarely occurs in children. It is more difficult to diagnose in patients with skin rash as the first manifestation. The diagnosis and treatment of this case reflects the important role of multidisciplinary cooperation in the diagnosis and treatment of difficult and rare diseases.