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 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.

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 explore the effect of low levels of calcium on the biological characteristics of ameloblasts. 【Methods】 Rat primary ameloblasts were cultured in standard DMEM medium. After five days they were identified by RT-PCR and immunohistochemistry. Then 0, 0.6 and 1.2 mmoL/L Ca²⁺ and 100 mL/L fetal bovine serum were added into DMEM medium without calcium. After 48 hours, the cell morphology was observed by inverted microscope. The proliferation and apoptosis of cells were separately examined by MTT and AnnexinV-PI. Real-time quantitative PCR was used to detect the expression levels of amelogenin and KLK4 mRNA. 【Results】 After Five days in standard DMEM medium, the cells were shaped like the paving pattern. RT-PCR showed that both amelogenin and KLK4 were expressed in the cells. Immunohistochemical staining showed that most cells had positive staining for amelogenin. After 48 hours of calcium intervention, some cells in 1.2 mmoL/L Ca²⁺ group had higher nuclear density and poor light transmittance, and more high columnar cells could be observed in 1.2 mmoL/L Ca²⁺ group than those in 0 and 0.6 mmoL/L Ca²⁺ groups. With the decrease in calcium concentration in the medium, MTT showed that the proliferation activity of ameloblasts reduced (P<0.01). Annexin V-PI showed that the percentage of apoptotic cells decreased, and there was a significant difference between 1.2 mmoL/L and 0 mmoL/L Ca²⁺ groups (P<0.05). Real time-PCR showed that the expressions of amelogenin and KLK4 mRNA reduced (P<0.01). 【Conclusion】 Low-level calcium may inhibit the differentiation of ameloblasts, thereby affecting the formation of enamel mineralization.

Objective:To determine whether amoxicillin had an effect on the enamel mineralization of SD rats.Methods:Eighteen SD rats were randomly divided into three groups. The rats in the control group were given distilled water. The rats in two experimental groups were administered 50 or 100 mg/kg amoxicillin by intragastric administration from day 3 to day 17 after birth. The general condition, the structure of liver and kidney, the enamel surface changes of mandibular first molars and incisors were observed. The changes of Ca/P ratio on enamel surface were analyzed by X-ray energy dispersive spectrometer (EDS). The surface morphology after phosphoric acid treatment was examined by scanning electron microscopy (SEM). Histological changes in the ameloblasts of mandibular incisors were analyzed by hematoxylin and eosin (HE) staining.Results:Compared with the control group, the general conditions as well as liver and kidney structures of SD rats in 50 and 100 mg amoxicillin groups had no significant differences. There was no obvious chalky changes on the first mandibular molars of SD rats in each group. All the incisors in 50 and 100 mg groups showed different degrees of chalkiness in the labial incisal 1/3 enamel. X-ray EDS analysis showed that the Ca/P ratios of occlusal and incisal 1/3 enamel in 50 and 100 mg groups (occlusal 1/3 of mandibular first molars: 1.51±0.03 and 1.52±0.02, incisal 1/3 of mandibular incisors: 1.46±0.01 and 1.43±0.01) was significantly lower than that in the control group (occlusal 1/3 of mandibular first molars: 1.67±0.41, incisal1/3 of mandibular incisors: 1.73±0.07) ( P<0.05). However, there was no significant differences in the cervical 1/3 Ca/P ratio of mandibular first molars and incisors among the three groups (mandibular first molars: 1.56±0.04 for control group, 1.59±0.05 for 50 mg group and 1.57±0.04 for 100 mg group; incisors: 1.52±0.02 for control group, 1.47±0.01 for 50 mg group and 1.51±0.03 for 100 mg group) ( P>0.05). SEM observation showed that the enamel rods of the first molars and incisors in the 50 and 100 mg group varied in size and arranged disorderly. The spaces between the enamel rods were larger than that in the control group and some areas even appeared large pits. HE staining showed that the gaps between ameloblasts in 50 and 100 mg groups were significantly wider than that in the control group. Conclusions:Intake of amoxicillin during the period of enamel development of SD rats might affect enamel mineralization.

Objective: To detect the diffusional kurtosis imaging (DKI) parameters of patients with Alzheimer's disease (AD), and evaluate the inner link of DKI parameters with cognitive function and serum nerve injury index. Methods: 78 patients who were first diagnosed with AD in our hospital between December 2015 and January 2018 were enrolled in AD group, and 50 healthy volunteers who had physical examination in our hospital during the same period were enrolled in normal control group. The corpus callosum DKI parameters [mean kurtosis (MK), axial kurtosis (AK) and radial kurtosis (RK)] values, Mini-Mental State Examination (MMSE) score as well as serum nerve damage indexes [β amyloid 1-42 (Aβ1-422), S100B protein (S100B) and brain-derived neurotrophic factor (BDNF)] were compared between the two groups of subjects. Pearson test was used to evaluate the correlation of DKI parameters with MMSE score as well as serum nerve injury index in patients with AD. Results: MK, AK and RK levels in AD group were lower than those in normal control group; MMSE score was lower than that of normal control group; serum Aβ1-42 and S100B contents were higher than those of normal control group while BDNF content was lower than that of normal control group (P<0.05). Correlation analysis revealed that the MK, AK and RK values in AD patients were directly correlated with the MMSE score as well as Aβ1-42, S100B and BDNF levels (P<0.05). Conclusions The corpus callosum DKI parameter levels decrease in AD patients, and the specific levels are closely related to the severity of cognitive function and nerve injury, which may be one of the effective methods for early assessment of AD condition.

Objective:To establish a predictive model of lateral lymph node metastasis in patients with papillary thyroid carcinoma(PTC), and further to compare the diagnostic efficiency of this model with the suspected abnormal lymph node thyroglobulin in fine-needle aspirate fluid (FNA-Tg) for lateral lymph node metastasis.Methods:The preoperative clinical and ultrasonographic data of 110 patients (257 lymph nodes) who underwent PTC cervical lymph node dissection were retrospectively analyzed. According to the postoperative pathological results, they were divided into lateral lymph node metastasis and non-metastasis group. Regression analysis was used to screen out independent risk factors affecting lateral lymph node metastasis and establish a predictive model. The ROC curve was used to evaluate the diagnostic efficacy and the best diagnostic cut-off point.Results:Prediction model: Logit( P)=-2.987+ 2.189(S/L ratio of lymph nodes)+ 1.748(hilum absent)+ 2.030(hyperechoic)+ 1.849(vascular abnormalities). The sensitivity, specificity, accuracy and AUC of the prediction model in the diagnosis of lateral lymph node metastasis were 92.1%, 83.9%, 87.9% and 0.929, respectively. The Homser-Lemeshow goodness of fit test showed that the Logistic model has a good fitting effect. The sensitivity, specificity, accuracy, and AUC of FNA-Tg in the diagnosis of lateral lymph node metastasis were 87.4%, 95.4%, 90.3% and 0.968, respectively. The sensitivity, specificity, accuracy, and AUC of the combined diagnosis of the predictive model and FNA-Tg were 92.9%, 96.9%, 94.2% and 0.989, respectively. Conclusions:The model has a good predictive value for PTC cervical lymph node metastasis. Combined with FNA-Tg, it can improve its diagnostic efficiency and provide more valuable information for the decision-making of clinical surgical procedures.

Objective To explore the sonographic features and clinical factors associated with skip metastasis in papillary thyroid carcinoma (PTC).Methods We reviewed 276 PTC cases pathologically confirmed after operation in our hospital,analyzing the ultrasonographic features and clinical characteristics of primary site and risk factors of skip metastasis.Results The rate of skip metastasis was 16.6％,and level Ⅱ or level Ⅲ were the most common area.In univariate analysis,skip metastasis was associated with tumor location,tumor maximum size,and calcification.Multivariate analysis showed the primary tumor location in the upper portion,tumor size ≤ 10 mm,and microcalcification were independent predictive factors for skip metastasis.Conclusions In papillary thyroid carcinoma,skip metastases rate is high when primary tumor location in the upper portion,tumor size ≤ 10 mm,and there is microcalcification.

Objective To study the correlation of whole brain apparent diffusion coefficient (ADC) with neurological impairment,homocysteine (Hcy) metabolism and oxygen free radical generation in patients with Alzheimer's disease (AD).Methods The patients diagnosed as AD in Xuanwu Hospital of Capital Medical University from March 2014 to December 2017 were selected as AD group and healthy persons as control group.The ADC of whole brain was calculated after magnetic resonance scanning.The degree of neurological impairment was evaluated by Montreal Cognitive Function Assessment Scale (MoCA).The levels of Hcy metabolic index and oxygen free radical production were measured after collecting serum.Results ADC values of AD group in parietal lobe,frontal lobe,temporal lobe and occipital lobe were not significantly different from those of control group (P ＞ 0.05).The ADC value of hippocampus as well as serum Hcy,malondialdehyde (MDA) and nitric oxide (NO) contents were significantly higher than those of control group,while MoCA score as well as folic acid (FA),vitamin B12 (VitB12),superoxide dismutase (SOD) and glutathione peroxidase (GSH) contents in serum were significantly lower than those of control group (P ＜ 0.05);ADC value of hippocampus in AD patients was negatively correlated with MoCA score as well as serum FA,VitB12,SOD and GSH contents,and positively correlated with serum Hcy,MDA and NO contents.Conclusions The increased ADC value of hippocampus in AD patients was related to the cognitive function injury,Hcy metabolism disorder and excessive generation of oxygen free radicals.

Objective: To investigate the occurrence of occult carcinoma in contralateral lobes based on the ultrasonic features of unilateral papillary thyroid carcinoma. Methods: The study included 202 consecutives cases of unilateral papillary thyroid carcinoma with benign nodules in the contralateral lobe identified by preoperative ultrasound or fine-needle aspiration from June 2014 to December 2015. All patients received total thyroidectomies, and with postoperative pathological examination they were divided into two groups, one including 60 cases with positive occult cancer and another one consisting of 142 cases with negative occult cancer. Univariate and multivariate analyses were performed to analyze the sonographic features of unilateral papillary thyroid carcinoma relevant to the occurrence of occult carcinoma in the contralateral nodules. Results: Univariate analysis indicated occult carcinoma in the contralateral lobes was associated with Hashimoto's thyroiditis(χ²=3.955, P=0.047), unclear border (χ²=4.375, P=0.036)and multifocality in the ipsilateral(χ²=7.375, P=0.007), but not with tumors maximum size, location, A/T, shape, internal structure, internal echo, acoustic halo, calcification, capsular invasion and blood flow signal in the lobe with carcinoma on another side. Multivariate analysis showed unclear border (OR=2.727, P=0.010) and multifocality in the ipsilateral(OR=2.807, P=0.005)of carcinoma were independent predictive factor for contralateral occult PTC. Conclusions Unclear border and multifocality of PTC in the ipsilateral were closely relevant to the occurrence of occult carcinoma in the contralateral nodules.