High C-reactive protein to lymphocyte ratio predicts mortality outcomes of patients with severe fever with thrombocytopenia syndrome: A multicenter study in China.

Severe fever with thrombocytopenia syndrome (SFTS) is a life-threatening infectious disease caused by the SFTS virus (SFTSV). This study aimed to evaluate the predictive power of C-reactive protein to lymphocyte ratio (CLR) and establish an early-warning model for SFTS mortality. We retrospectively analyzed hospitalized SFTS patients in six clinical centers from May 2011 to 2022. The efficacy of CLR prediction was evaluated by the receiver operating characteristic (ROC) analysis. A nomogram was established and validated. Eight hundred and eighty-two SFTS patients (median age 64 years, 48.5% male) were enrolled in this study, with a mortality rate of 17.8%. The area under the ROC curve (AUC) of CLR was 0.878 (95% confidence interval [CI]: 0.850-0.903, p < 0.001), which demonstrates high predictive strength. The least absolute shrinkage and selection operator regression selected seven potential predictors. Multivariate logistic regression analysis determined three independent risk factors, including CLR, to construct the nomogram. The performance of the nomogram displayed excellent discrimination and calibration, with significant net benefits in clinical uses. CLR is a brand-new predictor for SFTS mortality. The nomogram based on CLR can serve as a convenient tool for physicians to identify critical SFTS cases in clinical practice.

Differences in the Clinical Manifestations and Mortality of Systemic Lupus Erythematosus Onset in Children and Adults: A Systematic Review and Meta-Analysis.

INTRODUCTION: The aim of this study was to assess the differences between childhood-onset and adult-onset systemic lupus erythematosus (cSLE and aSLE) for clinical manifestations and mortality using a meta-analytic approach. METHODS: The PubMed, EMBASE, and the Cochrane library were searched for eligible studies published between January 1982 and March 2021. The odds ratio (OR) with 95% confidence interval was used to calculate the pooled effect estimates using the random-effects model. RESULTS: Thirty-four studies involving 21,946 SLE patients were included. cSLE was associated with an increased risk of malar rash (OR: 1.64; p < 0.001), ulcers/mucocutaneous involvement (OR: 1.22; p = 0.039), general neurological involvement (OR: 1.52; p < 0.001), seizures (OR: 1.92; p < 0.001), general renal involvement (OR: 2.08; p < 0.001), proteinuria (OR: 1.35; p = 0.015), urinary cellular casts (OR: 1.67; p = 0.047), fever (OR: 2.31; p < 0.001), anemia (OR: 1.91; p < 0.001), thrombocytopenia (OR: 1.41; p < 0.001), leucopenia (OR: 1.57; p = 0.017), lymphadenopathy (OR: 2.40; p < 0.001), and cutaneous vasculitis (OR: 1.72; p = 0.001) as compared with aSLE. Moreover, cSLE versus aSLE was associated with a reduced risk of articular manifestations (OR: 0.63; p = 0.001), pulmonary involvement (OR: 0.54; p = 0.001), and pleuritis (OR: 0.61; p < 0.001). There were no significant differences between cSLE and aSLE for mortality risk (OR: 1.20; p = 0.203). CONCLUSION: We found that certain clinical manifestations of SLE are different in cSLE and aSLE. Moreover, the mortality risk of cSLE and aSLE was not significantly different.

Analysis of five cases of monogenic lupus related to primary immunodeficiency diseases.

OBJECTIVE AND DESIGN: We studied five cases of PID-related monogenic lupus to explore the characteristics. MATERIAL OR SUBJECTS: Among 42 cases of PID patients between 2017-2020, 5 patients were diagnosed as PID-related monogenic lupus, including 2 males and 3 females, with age range from 2 years 3 months to 13 years old. TREATMENTS: DMARDs, biological agents and stem cell transplantation were used to treat different patients. METHODS: We collected the clinical observation indicators, auxiliary examination and treatment of the five patients. RESULTS: Patient 1 was diagnosed with monogenic lupus secondary to severe combined immunodeficiency and received prednisone and methotrexate treatment. Patient 2 was diagnosed with monogenic lupus secondary to activated phosphoinositide 3-kinase δ syndrome. Allogeneic stem cell transplantation was conducted. Patient 3 was diagnosed with monogenic lupus secondary to RAS-associated lymphoproliferative disease. The child was treated with prednisone and rituximab. Patient 4 was diagnosed with monogenic lupus secondary to PSTPIP1-associated myeloid-related proteinaemia inflammatory syndrome. The child was given methylprednisolone, methotrexate, and infliximab. Patient 5 was diagnosed with monogenic lupus secondary to A20 haploinsufficiency. The child was treated with methylprednisolone and infliximab. CONCLUSIONS: Multiple PIDs can lead to monogenic lupus. Different PID-related monogenic lupus has different suitable targeted drugs.

The quality of life in Chinese juvenile idiopathic arthritis patients: psychometric properties of the pediatric quality of life inventor generic core scales and rheumatology module.

BACKGROUND: Juvenile idiopathic arthritis (JIA) may seriously affects patients' quality of life (QoL), but it was rarely focused and studied in China, so we explore JIA children's QoL using Chinese version of the PedsQL4.0 Generic Core and PedsQL3.0 Rheumatology Module scale, and analyzed the psychometric properties of these two Scales among Chinese JIA children. METHODS: We recruited 180 JIA patients from Children's Hospital Affiliated to Capital Institute of Pediatrics and Hebei Yanda Hospital from July 2018 to August 2019. The questionnaires include information related on JIA, PedsQL4.0 generic core and PedsQL3.0 Rheumatology Module scales. According to the disease type, onset age of and course of JIA, we divided them into different groups, then compared the QoL status among different groups. Moreover, we analyzed the reliability and validity of these two scales in these 180 JIA children. RESULTS: The mean score of PedsQL4.0 generic core scale on these 180 patients was 82.85 ± 14.82, for these in active period was 72.05 ± 15.29, in remission period was 89.77 ± 9.23; the QoL score of systemic, polyarticular and oligoarticular JIA patients were 77.05 ± 19.11, 84.33 ± 12.46 and 87.12 ± 10.23. The mean score of PedsQL3.0 Rheumatology Module scale on 180 patients was 91.22 ± 9.45, for these in active period was 84.70 ± 11.37, in remission period was 95.43 ± 4.48; the QoL score of systemic, polyarticular and oligoarticular JIA patients were 89.41 ± 11.54, 89.38 ± 10.08 and 93.71 ± 6.92. In the PedsQL 4.0 Generic Core scale, the α coefficients of total scale and almost every dimension are all greater than 0.8 except for the school activity dimension of 0.589; the correlation coefficients of 22 items' scores (total 23 items) with the scores of dimensions they belong to are greater than 0.5 (maximum value is 0.864), and the other one is 0.406. In PedsQL3.0 Rheumatology Module scale, except for the treatment and worry dimensions of 0.652 and 0.635, the α coefficients of other dimensions and the total scale are all greater than 0.7; the correlation coefficients of all items' score were greater than 0.5 (the maximum is 0.933, the minimum is 0.515). CONCLUSIONS: The QoL of Chinese JIA children is worse than their healthy peers, these in active period and diagnosed as systemic type were undergoing worst quality of life. The reliability and validity of PedsQL 4.0 Generic Core and PedsQL3.0 Rheumatology Module scale in Chinese JIA children are satisfactory, and can be used in clinical and scientific researches.

Kinesin family member 2C promotes hepatocellular carcinoma growth and metastasis via activating MEK/ERK pathway.

The current work was intended to explore the function and mechanism of Kinesin family member 2C (KIF2C) in hepatocellular carcinoma (HCC). In this study, KIF2C expression was at a high level in HCC and indicated poor prognosis. Silencing KIF2C significantly suppressed the proliferation, migration, and invasion in HCC cells. Furthermore, silencing KIF2C markedly decreased the expression of Snail, Vimentin, p-MEK, and p-ERK, but increased E-cadherin expression in HCC cells. Moreover, we also found that MEK/ERK inhibitor U0126 could enhance the impact on cell proliferation, migration, and invasion induced by silencing KIF2C in HCC. On the contrary, MEK/ERK activator PAF could weaken the impact induced by silencing KIF2C in HCC. Thus, our findings indicate that KIF2C can promote the proliferation, migration, and invasion by activating MEK/ERK pathway in HCC.

Association between STAT4 polymorphisms and the risk of juvenile idiopathic arthritis in Han Chinese populations.

OBJECTIVES: Previous studies have demonstrated a potential role of STAT4 polymorphisms in increased juvenile idiopathic arthritis (JIA) risk in Caucasian populations; however, their role remains unclear in Han Chinese populations. We aimed to investigate single nucleotide polymorphisms (SNPs) of STAT4 and their role in JIA in Han Chinese populations. METHODS: This study included 205 JIA cases and 267 healthy controls. MassArray high-throughput DNA analyser and mass spectrometry were used to analyse 16 STAT4 SNP sites. The relationship between these SNPs and JIA risk was calculated using multiple logistic regressions. RESULTS: The G allele of rs11893432 was associated with an increased risk of JIA (odds ratio [OR]: 1.73; 95% confidence interval [CI]: 1.03-2.88; p=0.037). This relationship was observed in oligoarticular JIA (OR: 2.75; 95% CI: 1.29-5.83; p=0.026), and not in polyarticular JIA or systemic JIA. The GG motif was significantly correlated with oligoarticular JIA risk,compared to the CC+CG motif (OR: 1.88; 95% CI: 1.06-3.32; p=0.034). The C allele of rs1018981 and the A allele of rs10931481 were associated with a greater risk of polyarticular JIA (C allele: [OR: 7.82; 95% CI: 1.06-57.74; p=0.044]; A allele: [OR: 2.86; 95% CI: 1.23, 6.65; p=0.039). CONCLUSIONS: The G allele of rs11893432 was significantly associated with JIA risk, particularly oligoarticular JIA, in Han Chinese populations. SNPs at rs1018981 and rs10931481 were correlated with higher risk of polyarticular JIA.

Noonan syndrome and Noonan-like syndrome with loose anagen hair: rare phenotypes may emerge during follow-up.

Background: Noonan syndrome (NS) and Noonan-like syndrome with loose anagen hair (NS/LAH) are neurodevelopmental syndromes resulting from germline mutations in genes that participate in the rat sarcoma/mitogen-activated protein kinases (RAS/MAPK) pathway. The aim of this retrospective study was to describe common and rare manifestations of NS and NS/LAH. Methods: We collected and analyzed clinical and genetic data from 25 patients with NS and NS/LAH. Results: The patients' median age was 6.3 years (range, 1-13 years), and the male-to-female ratio was 18:7. In total, 19 patients had NS caused by a mutation in PTPN11. Another causative gene was found in six patients, including two patients with a SHOC2 mutation, one patient with a KRAS mutation, one patient with an LZTR1 mutation, one patient with a BRAF mutation, and one patient with a PPP1CB mutation. Short stature was detected in 100% of the patients. This study provides an overview of the clinical features of NS, including unique facial features, short stature, congenital heart defects, and other manifestations. Notably, systemic lupus erythematosus (SLE) was found in two SHOC2-positive patients. One patient had a posterior urethral valve, which is very rare in NS patients. Conclusions: Our study identified several clinical features that were previously poorly related to NS, including SLE. We concluded that SHOC2-related NS is associated with a particularly high risk of SLE, which may have a significant impact on quality of life, and a posterior urethral valve is a novel phenotype. These findings could be helpful in enhancing the understanding of the clinical spectrum of NS.

Children systemic lupus erythematosus-associated pancreatitis.

OBJECTIVE: To early recognise and improve the prognosis of children systemic lupus erythematosus (cSLE)-associated pancreatitis by summarising and analysing clinical features and prognosis data from 12 cases. METHODS: Retrospective analysis of clinical data from 12 cases of cSLE-associated pancreatitis diagnosed and treated from January 2016 to December 2021 at hospitals such as Children's Hospital of Capital Institute of Paediatrics. RESULTS: The median SLEDAI-2K score for disease activity was 18.00 (range 12.25-21.00) in the case group and 10.00 (range 7.00-18.00) in the control group, with a statistically significant difference (P < 0.05) between the two groups. The case group had a higher proportion of abdominal pain, vomiting, abdominal distension, pleural effusion, Raynaud's phenomenon (RP), splenic infarction, and concurrent macrophage activation syndrome (MAS) than the control group, with a statistically significant difference (P < 0.05). Serum ferritin (SF), alanine transaminase (ALT), aspartate transaminase (AST), lactate dehydrogenase (LDH), amylase, and increased 24-h urine protein levels were statistically different between the two groups (P < 0.05); platelet counts (PLT) reduction was also statistically different (P < 0.05). The case group had a higher proportion of methylprednisolone pulse therapy, cyclophosphamide pulse therapy during remission induction, and therapeutic plasma exchange than the control group, with a statistically significant difference (P < 0.05) between the two. CONCLUSION: CSLE-associated pancreatitis has a high fatality rate. The presence of RP, splenic infarction, pleural effusion, and MAS warrants attention from clinicians regarding the possibility of pancreatitis. Once pancreatitis is detected, the primary disease needs active treatment for better prognosis.

Validity of A Novel Simulator for Percutaneous Transforaminal Endoscopic Discectomy.

BACKGROUND: Percutaneous transforaminal endoscopic discectomy (PTED) has steep learning curves and a high incidence of complications, but currently, efficient and economical training methods are lacking. This study aimed to validate a novel simulator for PTED. METHODS: The simulated PTED included puncturing and establishing the working channel (PEWC) and endoscopic discectomy, with the PEWC being the tested module. Eleven experts and 21 novices were included and introduced to the simulator and tasks; all participants completed the PEWC. Outcomes included: total operation time, number of fluoroscopy for positioning the working sheath, number of spinal risk region invasion, Global Rating Scale (GRS) and a modified GRS, etc. The Mann-Whitney U test was used to compare 2 groups. Spearman's correlation coefficient analyzed continuous variables. RESULTS: Experts outperformed novices in total operation time (P = 0.001), requiring fewer number of fluoroscopies for positioning the working sheath (P = 0.003). Additionally, experts had a lower number of spinal risk region invasions (P = 0.016) and higher scores on both the GRS (P < 0.001) and modified GRS (P < 0.001). PTED experience correlated with GRS scores (P = 0.001) and modified GRS (P < 0.001). The overall realism scored a median of 4 (3.75-5), and educational value had a median of 4 (range 3-5). CONCLUSIONS: This study demonstrates the validity of the novel simulator, revealing significant associations between PTED experience and performance metrics in a simulated PEWC setting. Furthermore, the PEWC module also offers a good realistic design and high education value according to experts.

Increased cTnI Predicts Early Death in Patients with Severe Fever with Thrombocytopenia: A Multicenter Study in North China.

Background: Myocardial injury is common in severe fever with thrombocytopenia syndrome (SFTS) patients. Currently, research on the prognostic value of cardiac troponin I (cTnI) for predicting the mortality of SFTS patients, especially death within 7 days is limited. Methods: Between May 2011 and October 2022, clinical and laboratory data on admission of consecutive SFTS cases were collected from six medical centres in China. The clinical endpoint was in-hospital all-cause death within seven days. Risk factors of myocardial injury and death were analysed using multivariable regression models. Prognostic models were established using Cox regression and performance of indicators was evaluated in terms of calibration, discrimination. Results: A total of 1379 laboratory-confirmed patients were enrolled, in which 686 subjects were included for analysis. The median age was 66 years, with 48.1% of male. Eighty-seven patients died within seven days and 396 patients diagnosed with myocardial injury during hospitalization. Non-survivors had significant higher levels of cardiac indices than survivors, including cTnI, aspartic transaminase (AST) and lactate dehydrogenase (LDH). Elevated levels of cTnI (HR = 1.058, 95% CI:1.032-1.085), AST (HR = 1.191, 95% CI:1.150-1.234) and LDH (HR = 1.019, 95% CI:1.009-1.029) predicted risk of early in-hospital mortality. cTnI model performed best, with area under curve of 0.850 (0.774-0.926) and concordance index of 0.842, respectively. Statistical differences were found between high and low levels of cTnI for mortality (P<0.001) using 0.35 ng/mL as the optimal cut-off. Conclusion: The risk of early in-hospital death can be predicted by cTnI. Clinical doctors should remind vigilant concerning the elevation of cardiac enzyme as soon as possible.

The Association Between Antidepressant Use and Drug-Induced Liver Injury: A Nationwide, Population-Based Case-Control Study in Taiwan.

BACKGROUND AND OBJECTIVE: The complex risk factors of liver injury have prevented the establishment of causal relationships. This study aimed to explore the effects of antidepressant class, cumulative days of medication exposure, presence of comorbidities, and the use of confounding drugs on the risk of antidepressant-induced liver injury. METHODS: The population-based case-control study sample included individuals registered on the Taiwan National Health Insurance Database between 2000 and 2018. Hospitalized patients with suspected drug-induced liver injury were considered as cases, while control subjects were matched 1:1 by age, gender, and index date (the first observed diagnosis of liver injury). Multivariable regression models were performed to evaluate the association between antidepressants and liver injury. RESULTS: The findings showed that antidepressant users exhibited a higher risk of liver injury (adjusted odds ratio [aOR] 1.16, 95% confidence interval [CI] 1.12-1.20), particularly those prescribed non-selective serotonin reuptake inhibitors (NSRIs; aOR 1.05; 95% CI 1.01-1.10), selective serotonin reuptake inhibitors (SSRIs; aOR 1.22; 95% CI 1.16-1.29), serotonin-norepinephrine reuptake inhibitors (SNRIs; aOR 1.18; 95% CI 1.13-1.24), and others (aOR 1.27; 95% CI 1.14-1.42). Moreover, cases exhibited a more significant proportion of antidepressant usage and longer durations of treatment compared with controls. The risk of liver injury was higher in the first 30 days of use across all classes of antidepressants (aOR 1.24; 95% CI 1.18-1.29). CONCLUSION: SSRIs or SNRIs are commonly used to treat depression and other psychological disorders, and consideration of their potential effects on the liver is essential.

Activated partial thromboplastin time predicts mortality in patients with severe fever with thrombocytopenia syndrome: A multicenter study in north China.

Background: Severe fever with thrombocytopenia syndrome (SFTS) is an emerging infectious disease with high lethality. This study aimed to determine whether prolonged activated partial thromboplastin time (APTT) predicted SFTS mortality. Methods: SFTS patients were enrolled from 6 hospitals in the north China. Subjects were divided into training cohort and 5 externally validation cohorts. The least absolute shrinkage and selection operator Cox regression model was performed to screen potential prognostic factors. Risk factors were analyzed using multivariable regression models. Prognostic models were established by Cox regression and random survival forest (RSF) methods, and evaluated regarding discrimination, validity and clinical benefit. Time-dependent receiver operating characteristic (ROC) curve was used to evaluate the predictive effectiveness of variables. Results: 1332 SFTS cases were included, in which 211 patients died. Six potential prognostic factors were screened, and pulse, breath, APTT and aspartic transaminase (AST) were independently associated with mortality in both training cohort (Yantai, N = 791) and external validation cohort (N = 541). APTT was steadily correlated with the fatality (HR: 1.039-1.144; all P < 0.01) in each five sub-validation cohorts (Dandong, Dalian, Tai'an, Qingdao and Beijing). RSF model with variables of APTT, AST, pulse and breath had considerable prognostic effectiveness, which APTT showed the highest prognostic ability with the area under the curve of 0.848 and 0.787 for 7-day and 14-day survival, respectively. Survival differences were found between high and low levels of APTT for mortality using 50s as the optimal cut-off. Conclusions: SFTS patients have prolonged APTT, which is an independent risk factor for fatality. APTT≥50s was recommended as a biomarker to remind physicians to monitor and treat patients more aggressively to improve clinical prognosis.

Pediatric Behçet's disease with cardiac valvular lesions: A case-based review.

Due to the insidious nature of pediatric cardiac Behçet's disease (BD), misdiagnosis or missed diagnosis occurred frequently. We described a female pediatric patient with BD with cardiac valvular involvement diagnosed at the age of 4 years with clinical symptoms, including aphthous ulcers, fever, perianal ulcers, and erythema nodosum, as well as significantly elevated inflammatory markers. Echocardiography revealed that previously absent aortic valve lesions developed later and gradually worsened. After being diagnosed with BD with cardiovascular involvement, the patient was treated with glucocorticoids, immunosuppressants, biologics, diuretics, and aortic valvuloplasty. At the time of the follow up, the patient was stable. A review of 13 publications was conducted, including 14 cases of cardiac involvement in pediatric BD.

Comparative efficacy of exercise training processes in improving nonalcoholic fatty liver disease: a systematic review and meta-analysis.

OBJECTIVES: This systematic review and meta-analysis aimed to evaluate the efficacy of exercise training in patients with nonalcoholic fatty liver disease (NAFLD). METHODS: We searched PubMed, Cochrane Library, Web of Science, and Embase for relevant research from January 2001 to December 2021. The efficacy of exercise training was analyzed. RESULTS: A total of 21 articles, involving 1733 patients, were included. Exercise training, including resistance training, aerobic exercise training, and high-intensity training, showed the efficacy in reducing weight (MD = 3.46, 95% CI [1.94, 4.98]), BMI (MD = 0.89, 95% CI [0.17, 1.61]), and ALT (MD = 6.66, 95% CI [3.27, 10.04]) and AST (MD = 3.14, 95% CI [0.35, 5.93]) levels in patients with NAFLD. When the exercise training lasted for ≥ 20 weeks, the total cholesterol (TC) (MD = 0.13, 95% CI [0.04, 0.22]), triglyceride (TG) (MD = 0.29, 95% CI [0.12, 0.47]), and blood glucose (GLU) (MD = - 0.18, 95% CI [0.10, 0.26]) levels significantly reduced. Compared with the exercise training group, the exercise training combined with probiotics group showed more efficiency in reducing the ALT, AST, TG, and TC levels. However, the exercise training combined with a hypoglycemic agent group showed no obvious efficiency compared with the exercise training group. CONCLUSION: Exercise training can improve NAFLD. The improvement was more obvious when exercise was performed for ≥ 20 weeks. Probiotics may enhance the efficiency of exercise training.

Synthesis of WO3@WS2 core-shell nanostructures via solution-based sulfurization for improved performance of water splitting.

High light absorption capacity and excellent charge transportation are significant for superior water-splitting performance. Here, WO3/WS2 core-shell nanowire arrays were fabricated using a two-step hydrothermal method. The crystal phase, morphology, crystal structure, chemical composition, and optical properties were characterized using XRD, SEM, TEM, XPS, and UV-vis spectroscopy. Consequently, the photocurrent density of the as-prepared WO3/WS2 photoanode was 0.91 mA cm-2 (at 1.23 V vs. RHE), which showed a 112% increase compared to that with pristine WO3. The enhanced photoelectrochemical performance, we believe, was due to the promoted light response and improved separation as well as transportation at the WO3/WS2 interface.

Paediatric autoimmune diseases with ELANE mutations associated with neutropenia.

OBJECTIVE: To explore the clinical characteristics of autoimmune diseases in children with ELANE mutations. METHODS: Three cases of children with ELANE mutations manifesting as autoimmune diseases, who were under treatment from April 2020 to May 2021, were retrospectively analysed. RESULTS: Among the three children, two were boys aged 15 years and 22 months (cases 1 and 3) respectively, and the other one was a 22-month-old girl (case 2). All the cases had recurrent infections. Case 1 presented with cyclic neutropenia and systemic lupus erythematosus (SLE). Case 2 presented with severe neutropenia and autoimmune haemolytic anaemia (AHIA). Case 3 presented with severe neutropenia and anti-neutrophil cytoplasm antibodies (ANCA)-associated small vasculitis. Genetic tests showed that they all had heterozygous mutations in the ELANE gene. Case 1 was treated with methylprednisolone and hydroxychloroquine sulphate for 2 years, making neutrophil level return to normal. Case 2 received allogeneic hematopoietic stem cell transplantation and has stopped taking antibiotics, steroids and all the immunosuppressors. Case 3 received subcutaneous injections of granulocyte colony-stimulating factor, oral prednisone and cyclophosphamide. The boy in case 3 has been followed up for one year, and his absolute neutrophil count has increased to 1.56 × 109/L. CONCLUSION: Patients with ELANE mutations, combined with autoimmune diseases, may have recurrent infections. Disease-modifying antirheumatic drugs (DMARDs) are effective for autoimmune diseases. Autoimmune diseases with ELANE mutations associated with neutropenia can be cured through allogeneic hematopoietic stem cell transplantation.

Correlation between CT Score and KL-6: A Severity Assessing in Juvenile Dermatomyositis Associated Interstitial Lung Disease.

Background: There is no radiological measurement to estimate the severity of pediatrics juvenile dermatomyositis (JDM) with interstitial lung disease (ILD). We validated the effectiveness of CT scoring assessment in JDM patients with ILD. Aim: To establish a CT scoring system and calculate CT scores in JDM patients with ILD and to determine its reliability and the correlation with Krebs von den Lungen-6 (KL-6). Methods: The study totally enrolled 46 JDM-ILD patients and 16 JDM without ILD (non-ILD, NILD) patients. The chest CT images (7.0 ± 3.6 years; 32 male and 30 female) were all analyzed. CT scores of six lung zones were retrospectively calculated, included image pattern score and distribution range score. Image pattern score was defined as follows: increased broncho-vascular bundle (1 point); ground glass opacity (GGO) (2 points); consolidation (3 points); GGO with bronchiectasis (4 points); consolidation with bronchiectasis (5 points); and honeycomb lung (6 points). Distribution range score was defined as no infiltrate (0 point); <30% (1 point); 30%-60% (2 points); and ≥60% (3 points). Two pediatric radiologists reviewed all CT images independently. The ROC curve was established, and the optimal cutoff score for severity discrimination was set. Results: The agreement between two observers was excellent, and the ICC was 0.930 (95% CI 0.882-0.959, p < 0.01). CT score and KL-6 level had a positive linear correlation (r = 0.784, p < 0.01). However, the correlation between CT scores of different lung zone and KL-6 level was different. The KL-6 cut off level suggested for JDM with ILD was 209.0 U/ml, with 73.9% sensitivity and 87.5% specificity, and the area under curve was (AUC) 0.864 (p < 0.01). Conclusion: The CT scoring system we established, as a semiquantitative method, can effectively evaluate ILD in JDM-PM patients and provide reliable evidence for treatment.

Clinical characteristics and risk factors of coronary artery lesions in chinese pediatric Takayasu arteritis patients: a retrospective study.

BACKGROUD: To summarize the clinical characteristics and identify the risk factors for pediatric Takayasu arteritis (TAK) with coronary artery lesions (CALs). METHODS: Clinical data of pediatric TAK patients in our center were retrospectively assessed. Independent risk factors for CALs were identified using multivariate logistic regression analysis. Survival analysis was used to compare differences in survival rates between the groups. RESULTS: Among the 66 pediatric TAK cases, the incidence of accompanying CALs was 39.4%. In the CAL group, 19 (73.1%) cases started within 36 months. None of the patients had symptoms of angina or ischemia on electrocardiogram (ECG), the CALs were detected using coronary ultrasound. The CALs most commonly were the left main and right coronary arteries. The lesions were mostly small or middle coronary artery aneurysms; some children may have giant coronary aneurysmal dilations, thrombosis and heart failure. The age of onset and symptom onset to diagnosis in TAK patients with CAL were lower than those in TAK patients without CAL(P < 0.005). TAK patients with CAL had significantly higher CRP,WBC, PLT,TNF-α and IL-2R levels (P < 0.05), lower HGB (P = 0.01), lower rate of renal artery stenosis (RAS) (P = 0.009). In multivariate logistic regression, the risk factors for pediatric TAK combined with CAL included the age of TAK onset (OR = 0.9835, 95% CI: 0.9710-0.9946, P = 0.006) and RAS (OR = 0.1901, 95% CI: 0.0386-0.7503, P = 0.03). In addition, there was no significant difference in survival rates between the two groups after regular treatment. CONCLUSION: This study showed that the occurrence of CAL in pediatric TAK patients has a relatively more rapid clinical course, and a stronger inflammatory state at the time of diagnosis. The earlier the age of TAK onset and without RAS are more likely to cause CAL.

Clinical characteristics and prognosis of childhood-onset lupus mesenteric vasculitis as the initial presentation-a case-control study.

BACKGROUND: Lupus mesenteric vasculitis (LMV) as initial presentation is rare, especially in childhood-onset systemic lupus erythematosus (cSLE). It is a critical complication of lupus. At present, the research on cSLE with LMV as the initial presentation is few. The aim of this study was to analyze the clinical characteristics and prognosis of cSLE with LMV in the Chinese population, compared with non-LMV cSLE. METHODS: A retrospective case-controlled study was conducted on 55 cSLE patients between July 2018 and July 2021. The clinical data, laboratory findings, imaging, treatment, and follow-up data were collected and compared between the two groups of cSLE with LMV and non-LMV. Non-LMV cSLE patients were matched according to the age and sex of LMV patients. RESULTS: A total of 11 cSLE patients with LMV as the LMV group and 44 cSLE patients without LMV as the non-LMV group were included. The average age of onset was 12.55 ± 1.57 years old, the male-to-female ratio was 2:9, and high disease activity was observed in the LMV group. Abdominal pain was most common in LMV. Compared with the non-LMV, the percentage of abdominal pain, vomiting, abdominal distension, and diarrhea was higher, and gastrointestinal tract, serous cavity, kidney, and lung damage were higher in the LMV group (P < 0.05). In abdominal-enhanced CT, the percentage of intestinal wall thickening, peritoneal effusion, mesenteric vascular enhancement, hydronephrosis with ureteral dilatation, intestinal congestion, and gastric mucosa thickening in the LMV group were higher than those in the non-LMV group (P < 0.05). The percentage of receiving methylprednisolone pulse combined with cyclophosphamide pulse therapy in LMV was higher than in non-LMV. The clinical symptoms disappeared quickly, and there were no deaths in the LMV group. Compared with the non-LMV group, the 24-h urinary protein was higher, the complement C3 was lower, and the disease activity was higher in the LMV group (P < 0.05). CONCLUSIONS: LMV often occurs in 12 ~ 13-year-old girls with high disease activity of cSLE. Abdominal pain is the most common and more susceptible to damage to the kidney, serous cavity, and lung in cSLE with LMV. Methylprednisolone pulse combined with CTX pulse therapy is effective. After the treatment above, cSLE with LMV has a good prognosis, but the overall recovery is worse than non-LMV patients.

Clinical observations on infliximab treatment of infantile onset Takayasu arteritis.

BACKGROUND: There is insufficient evidence on the clinical effectiveness and safety of infliximab (IFX) treatment of Takayasu arteritis (TA) in infants. METHODS: We evaluated the therapeutic effectiveness and safety of IFX in a retrospective case series of 10 infantile TA patients. Observations included assessment of clinical symptoms, laboratory testing, and vascular imaging. RESULTS: Fever was the presenting symptom for 8 of 10 infants with TA. During acute episodes, leucocyte and inflammatory indices were significantly increased. Vascular imaging showed the most commonly involved arteries to be carotid arteries, abdominal aortas, and coronary arteries (9 cases, 90%). Two weeks after initiating IFX treatment, leukocyte and platelet counts decreased and hemoglobin levels increased. There were statistically significant clinical improvements 6 weeks after starting treatment compared with before treatment (p < 0.05). Inflammatory indices decreased 2 weeks after starting IFX treatment compared with before treatment (p < 0.05). Vascular lesions began to recover within 1.5-3 months of initiating IFX therapy, and involved vessels significantly recovered within 13 months. Some arteries remained stenotic, with intimal thickening and uneven lumen wall thicknesses. The only adverse event was a treatment-responsive allergic reaction during IFX infusion in one infant. CONCLUSIONS: Fever was the main manifestation of illness and was often accompanied by significantly increased inflammatory indices. IFX treatment was apparently effective and reduced or eliminated need for glucocorticoids. IFX had a reasonably good safety profile.