Objective:To explore the clinical characteristics, diagnosis and treatment strategies, and prognosis of pulmonary embolism (PE) complicating childhood rheumatic diseases.Methods:A retrospective case series study was performed on the demographic data, laboratory indicators, imaging features, treatment regimens, and follow-up data of 8 children with rheumatic diseases complicated by PE who were admitted to the Department of Rheumatology and Immunology, Capital Center for Children′s Health, Capital Medical University from January 2014 to October 2023.Results:Among the 8 children, there were 4 boys and 4 girls, with an age of 12.0 (7.5, 13.0) years. Among the primary diseases, there were 3 cases of systemic lupus erythematosus, 2 cases of Beh?et′s disease, 2 cases of Takayasu arteritis, and 1 case of antiphospholipid syndrome. All children developed PE during the active phase of the primary disease. PE was detected at the onset of the primary disease in 3 cases, and the median time from the diagnosis of the primary disease to the development of PE was 10.0 (6.0, 25.0) months in the remaining 5 cases. Fever was present in all 8 children, 4 cases were accompanied by chest tightness, dyspnea, etc., and 2 cases only presented with fever. Laboratory examinations revealed the following results: erythrocyte sedimentation rate was 42.0 (17.0, 78.0) mm/1 h, high-sensitivity C-reactive protein was 12.7 (2.6, 78.7) mg/L, white blood cell count was 9.6 (7.2, 18.7)×10 9/L; D-dimer was 2.3 (0.9, 6.2) mg/L; and hemoglobin was (109±16) g/L.Imaging examinations revealed that 5 cases had involvement of the bilateral lower pulmonary arteries, 5 cases had peripheral embolism, and 3 cases had central PE. Complications included 3 cases of deep vein thrombosis, 2 cases of intracranial venous sinus thrombosis, and 1 case of mild pulmonary hypertension.In terms of treatment, 7 cases received anticoagulation with heparin followed by warfarin. Immunomodulation was mainly based on glucocorticoids combined with immunosuppressants, and 4 cases were combined with biological agents. The follow-up time of 4.17 (1.75, 7.17) years, the time for complete absorption of PE was 10.5 (6.0, 18.0) months; all 8 children had no target events, with no recurrence or chronic thromboembolic pulmonary hypertension, and the pulmonary artery remodeling was good. Conclusions:PE complicating childhood rheumatic diseases is closely related to the activity of the primary disease. The clinical manifestations are insidious, with fever as the main symptom. Imaging examination is the key to diagnosis.Early adoption of heparin followed by warfarin anticoagulation and glucocorticoids combined with immunosuppressants and (or) biological agents to control the primary disease can achieve a favorable prognosis.

Objective:To explore the clinical characteristics, diagnosis and treatment strategies, and prognosis of pulmonary embolism (PE) complicating childhood rheumatic diseases.Methods:A retrospective case series study was performed on the demographic data, laboratory indicators, imaging features, treatment regimens, and follow-up data of 8 children with rheumatic diseases complicated by PE who were admitted to the Department of Rheumatology and Immunology, Capital Center for Children′s Health, Capital Medical University from January 2014 to October 2023.Results:Among the 8 children, there were 4 boys and 4 girls, with an age of 12.0 (7.5, 13.0) years. Among the primary diseases, there were 3 cases of systemic lupus erythematosus, 2 cases of Beh?et′s disease, 2 cases of Takayasu arteritis, and 1 case of antiphospholipid syndrome. All children developed PE during the active phase of the primary disease. PE was detected at the onset of the primary disease in 3 cases, and the median time from the diagnosis of the primary disease to the development of PE was 10.0 (6.0, 25.0) months in the remaining 5 cases. Fever was present in all 8 children, 4 cases were accompanied by chest tightness, dyspnea, etc., and 2 cases only presented with fever. Laboratory examinations revealed the following results: erythrocyte sedimentation rate was 42.0 (17.0, 78.0) mm/1 h, high-sensitivity C-reactive protein was 12.7 (2.6, 78.7) mg/L, white blood cell count was 9.6 (7.2, 18.7)×10 9/L; D-dimer was 2.3 (0.9, 6.2) mg/L; and hemoglobin was (109±16) g/L.Imaging examinations revealed that 5 cases had involvement of the bilateral lower pulmonary arteries, 5 cases had peripheral embolism, and 3 cases had central PE. Complications included 3 cases of deep vein thrombosis, 2 cases of intracranial venous sinus thrombosis, and 1 case of mild pulmonary hypertension.In terms of treatment, 7 cases received anticoagulation with heparin followed by warfarin. Immunomodulation was mainly based on glucocorticoids combined with immunosuppressants, and 4 cases were combined with biological agents. The follow-up time of 4.17 (1.75, 7.17) years, the time for complete absorption of PE was 10.5 (6.0, 18.0) months; all 8 children had no target events, with no recurrence or chronic thromboembolic pulmonary hypertension, and the pulmonary artery remodeling was good. Conclusions:PE complicating childhood rheumatic diseases is closely related to the activity of the primary disease. The clinical manifestations are insidious, with fever as the main symptom. Imaging examination is the key to diagnosis.Early adoption of heparin followed by warfarin anticoagulation and glucocorticoids combined with immunosuppressants and (or) biological agents to control the primary disease can achieve a favorable prognosis.

Objective To investigate the changes in the prevalence of Babesia microti infections, spleen morphology and proportions of splenic immune cells in BALB/c mice following intravenous and intraperitoneal injections, so as to provide insights into unraveling the immune regulatory mechanisms of Babesia infections. Methods Laboratory - maintained B. microti strains were prepared into whole blood samples with 10% prevalence of B. microti infection. A total of 75 BALB/c mice were randomly divided into three groups, including the normal control group, intravenous injection group, and intraperitoneal injection group, of 25 mice in each group. Mice in the intravenous and intraperitoneal injection groups were administered 100 μL of whole blood samples with 10% prevalence of B. microti infection, with the day of injection recorded as d0, and animals in the normal control group were given no treatments. Blood was sampled from mice in each group via the tail tip on d7, d14, d21, d28 and d35, and prepared into thin-film blood smears, and B. microti infection was observed in red blood cells. Five mice were randomly sampled from each group and sacrificed on d7, d14, d21, d28 and d35, and spleen was collected for measurement of spleen size and weight. In addition, splenic cells were isolated, and the proportions of CD3e⁺ T cells, CD45R⁺ B cells, CD49b⁺ nature killer (NK) cells, and F4/80⁺ macrophages were detected in CD45⁺ lymphocytes using flow cytometry. Results The prevalence of B. microti infection in the intravenous (22.80%) and intraperitoneal injection groups (44.82%) peaked on d7 (χ² = 8.141, P < 0.01) and then rapidly decreased, and no parasites were observed on d35. The longest mouse spleen length [(32.91 ± 2.20) mm] and width [(9.82 ± 0.43) mm], and the greatest weight [(0.78 ± 0.10) g] were found on d14 in the intravenous injection group, and the longest spleen length [(32.42 ± 3.21) mm] and width [(10.25 ± 0.73) mm], and the greatest weight [(0.73 ± 0.09) g] were seen in the intra-peritoneal injection group on d21, d7 and d14, respectively. There were significant differences among the intravenous injection group, intraperitoneal injection group and the normal control group in terms of spleen length (F = 10.310, P < 0.05), width (F = 9.824, P < 0.05), and weight (F = 10.672, P < 0.05) on d21, and the mouse spleen length, width and weight were all significantly greater in the intraperitoneal injection group than in the intravenous injection group (allP values < 0.05). The proportions of splenic CD3e⁺ T cells [(60.60 ± 6.20)% and (39.68 ± 7.62)%], CD45R⁺ B cells [(43.32 ± 2.08)% and (49.53 ± 4.90)%], CD49b⁺ NK cells [(6.88 ± 1.34)% and (7.71 ± 1.59)%], and F4/80⁺ macrophages [(2.21 ± 0.29)% and (3.80 ± 0.35)%] peaked on d14, d21, d21 and d14 in the intravenous and intraperitoneal injection groups, respectively. There were significant differences in the proportions of CD3e⁺ T cells (F = 16.730, P < 0.05) and F4/80⁺ macrophages (F = 15.941, P < 0.05) among the intravenous injection group, intraperitoneal injection group and normal control group on d14, and a higher proportion of CD3e⁺ T cells and a lower proportion of F4/80⁺ macrophages were detected in the intravenous injection group than in the intraperitoneal injection group (both P values < 0.01). There were significant differences among the intravenous injection group, intraperitoneal injection group and normal control group on d21 in terms of proportions of splenic CD3e⁺ T cells (F = 9.252, P < 0.05), CD45R⁺ B cells (F = 14.349, P < 0.05), CD49b⁺ NK cells (F = 13.436,P < 0.05), and F4/80⁺ macrophages (F = 8.180, P < 0.05), and a higher proportion of CD3e⁺ T cells and lower proportions of CD45R⁺ B cells and F4/80⁺ macrophages were detected in the intravenous injection group than in the intraperitoneal injection group (all P values < 0.01). In addition, there was a significant difference in the proportion of CD3e⁺ T cells among the intravenous injection group, intraperitoneal injection group and normal control group on d28 (F = 9.772,P < 0.05), and a lower proportion of CD3e⁺ T cells was found in the intravenous injection group than in the intraperitoneal injection group (P < 0.01). Conclusions Both intraperitoneal and intravenous routes are effective to induce B. microti infections in BALB/c mice, and the prevalence of B. microti infections is higher in BALB/c mice through the intraperitoneal route than through the intravenous route. Intraperitoneal and intravenous injections with B. microti cause diverse spleen morphologies and proportions of splenic immune cells in mice, indicating routes of B. microti infections cause different impacts on immune response mechanisms in mice.

Objective To observe the value of CT angiography(CTA)for classifying type of peripheral arteriovenous fistula(AVF)in children.Methods Totally 22 children with peripheral AVF were retrospectively enrolled,the type of AVF was classified with preoperative CTA,and therapeutic planning was developed.Taken digital subtraction angiography(DSA)findings during interventional therapy as gold standards,the efficacy of CTA for classifying type of AVF was analyzed.Results Among 22 cases,DSA detected 3 cases of type Ⅰ,13 cases of type Ⅱa and 6 cases of typeⅡb peripheral AVF,while 3 cases of type Ⅰ,14 cases of type Ⅱa and 5 cases of type Ⅱb peripheral AVF were classified based on CTA.CTA misdiagnosed 1 case of type Ⅱa as type Ⅱb,and 2 cases of type Ⅱb as type Ⅱa peripheral AVF,its accuracy of CTA for classifying type Ⅰ,Ⅱa and Ⅱb peripheral AVF was 100%(3/3),92.31%(12/13)and 66.67%(4/6),respectively.Among 22 cases,13 cases underwent interventional closure,2 cases underwent interventional therapy combined with surgery,while 3 cases did not receive relevant treatment due to drainage vein occlusion.One month after treatments,the symptoms of 19 cases who underwent treatment improved significantly,and no signs of AVF was found with re-examination of ultrasound nor CTA.Conclusion CTA was helpful to classifying type of peripheral AV in children,which could provide references for interventional therapy.

Objective To explore the biological characteristics associated with different subtypes and the response to immunotherapy by integrating multiomics analysis and artificial neural networks(ANN)to delineate the precise molecular subtypes of adrenocortical carcinoma(ACC)and establish a prognostic prediction model,in order to provide reference for the accurate prognosis assessment and individualized treatment of ACC.Methods The multiomics data of 44 Chinese ACC patients admitted to the Department of Urology,West China Hospital of Sichuan University during Jan.1,2012 and Dec.31,2022 were integrated,including genomic,transcriptomic and clinical features.Ten different clustering algorithms were employed for consensus clustering to identify robust molecular subtypes.The results were then incorporated into an ANN model to construct an ANN-driven prognostic index(ANPI)for patient stratification and survival prediction.Results Three distinct molecular subtypes(cancer subtypes,CS1-3)with significantly different prognoses were identified,among which CS1 exhibited the poorest survival outcomes.A set of 20 core genes was selected to form the basis of the ANPI model.ANPI effectively stratified patients into high-and low-risk groups:patients in the low-ANPI group had significantly better overall survival and exhibited"hot tumor"immune phenotypes,suggesting greater benefits from immunotherapy.In contrast,high-ANPI patients had worse prognoses and displayed"cold tumor"characteristics with weaker immunotherapy responses.Conclusion Our integrative multiomics analysis illustrated the molecular landscape of ACC in the Chinese population and uncovered the key immune-related features linked to clinical outcomes.The ANPI model demonstrated strong performance in prognostic prediction and immunotherapy response assessment,offering a valuable tool for precision oncology and clinical decision-making.

Common rheumatic musculoskeletal(MSK)diseases in children include juvenile idiopathic arthritis,juvenile dermatomyositis and chronic nonbacterial osteomyelitis/chronic recurrent multifocal osteomyelitis,with clinical manifestations and laboratory findings lack specificity.Imaging plays an important role in diagnosing and monitoring treatment efficacy of related diseases.Traditional localized imaging is difficult to comprehensively assess the site and extent of lesions.Whole-body MRI,as a noninvasive,radiation-free imaging technique capable of displaying entire body through one scanning,has significant advantages,especially in pediatric patients,yet the requirement for high-end equipment,the lack of standardized scanning protocols,and the need of sedation in young children,limit the application of whole-body MRI.It is of practical clinical urgency to raise clinical awareness of the advantages of whole-body MRI application and to propose expert consensus and guidelines for whole-body MRI in children through multi-neutral cooperation.

Objective:To investigate the value of 24 h urinary aldosterone(24 h-UAC) measurement by liquid chromatography-tandem mass spectrometry(LC-MS/MS) in the subtype classification of primary aldosteronism(PA).Methods:A total of 86 patients with PA, including 51 with unilateral primary aldosteronism(UPA) and 35 with bilateral primary aldosteronism(BPA), were enrolled in the Department of Endocrinology and Metabolism at West China Hospital between January 2018 and December 2022. Plasma aldosterone concentration(PAC), plasma renin concentration(PRC) and 24 h-UAC were measured by LC-MS/MS. 24-hour urinary electrolytes and 24-hour urinary creatinine(24 h-UCR) were also measured. The diagnostic value of 24 h-UAC in PA subtype classification was evaluated using receiver operating characteristic(ROC) curve analysis. Multivariate logistic regression analysis was conducted with PA subtypes as the dependent variable(UPA=1, BPA=0) to establish a diagnostic model for differentiating unilateral from bilateral lesions, and its performance was compared with published Chinese classification models. Results:There were no statistical differences between the UPA and BPA groups in terms of age, gender, BMI, systolic and diastolic blood pressure, 24 h urinary potassium, sodium, chloride, 24 h-UCR and PRC( P<0.05). The lowest plasma potassium level was significantly lower in the UPA group than in the BPA group, while PAC, 24 h-UAC, aldosterone-renin ratio(ARR), and 24 h-UAC/UCR were significantly higher( P<0.05). The detection rate of typical adenomas on imaging also showed a significant difference between the two groups( P<0.05). The area under the ROC curve(AUC) of 24 h-UAC for differentiating UPA from BPA was 0.829(95% CI 0.733-0.902), with an optimal cut-off value of 15.4 μg/24 h, yielding a sensitivity of 68.63% and a specificity of 88.57%( P<0.001). At a cut-off value of 24.5 μg/24 h, specificity reached 100%, with a sensitivity of 27.45%. Multivariate analysis indicated that a combined model incorporating 24 h-UAC, the lowest plasma potassium level, and imaging findings of typical adenomas significantly improved diagnostic accuracy for PA subtyping, achieving a specificity of 91.43%. Compared with the existing Chinese modified Küpers scoring model and CONPASS prediction model, this model demonstrated higher diagnostic efficiency, a lower missed diagnosis rate, and a misdiagnosis rate intermediate between the two. Conclusion:The 24 h-UAC in UPA patients is significantly higher than in BPA patients, making it a valuable marker for PA subtype classification. A predictive model combining 24 h-UAC, the lowest plasma potassium level, and imaging evidence of typical adenomas demonstrated high diagnostic accuracy for PA subtype classification and may provide valuable guidance for clinical decision-making.

Objective:To analyze the clinical characteristics of renal abscess in children and provide suggestions for early diagnosis and treatment.Methods:The clinical data including general information, laboratory data, imaging results, treatment and prognosis of 20 pediatric patients with renal abscess admitted to the Department of Nephrology, Capital Center for Children's Health Capital Medical University were analyzed retrospectively.Results:A total of 8 males and 12 females were enrolled. The age of onset was 3.0 (0.8, 9.0) years. All cases had fever. Six cases presented with abdominal pain, 6 cases had poor appetite, 5 cases had vomiting and 5 cases urinary tract irritation symptoms. Laboratory data showed elevated white blood cells 20.4 (17.4,26.3)×10 9/L, C-reactive protein 126 (77, 154)mg/L, erythrocyte sedimentation rate 60 (41,73) mm/1 h in 20 cases and procalcitonin 4.7 (1.2,33.5)μg/L in 10 cases. Totally 18 cases had pyuria. Urine culture was positive in 8 cases. Enterococcus faecium and Pseudomonas aeruginosa was detected in 2 cases. Klebsiella pneumoniae was found in 1 case after performing blood culture. Renal abscess was confirmed in all cases by doing contrast-enhanced CT scan, while only 9 cases with abscesses were identified by using renal ultrasound. There were 14 cases with renal abscess formation confirmed at onset by performing magnetic resonance imaging. Nine cases were accompanied with congenital anomalies of the kidney and urinary tract. All cases received conservative medical treatment. Intravenous broad-spectrum antibiotics were administered for 23 (14, 39) d initially, while the medication in 11 cases were upgraded to meropenem or imipenem. Oral antibiotics were continued for 23 (14, 28) d after discharge in all cases. Within 1 year of follow-up, except for 1 case of recurrence, the others had a favorable prognosis. Conclusions:Renal abscess should be suspected for children presenting with unexplained fever, vomiting, abdominal pain, elevated white blood cell count, C-reactive protein, erythrocyte sedimentation rate and pyuria. Ultrasonography is suitable for screening and follow-up, while CT or magnetic resonance imaging can be used to confirm the diagnosis. Conservative management with broad-spectrum antibiotics is effective and can be considered the first-line therapy for pediatric renal abscess.

Objective:To analyze the clinical characteristics of anti-melanoma differentiation associated gene 5 (MDA5) antibody-positive juvenile dermatomyositis (JDM) patients.Methods:A retrospective case-control study was conducted. The positive group included 18 children with anti-MDA5 antibody-positive JDM who were admitted to the Department of Rheumatology and Immunology at Capital Center for Children′s Health with Capital Medical University between January 2016 and January 2023. Another 36 children with anti-MDA5 antibody-negative JDM hospitalized during the same period were enrolled as the negative group. Based on the extent of pulmonary involvement and pulmonary CT scores, the MDA5-positive group was further divided into severe pulmonary involvement and non-severe pulmonary involvement subgroups. Chi-square test and Kruskal-Wallis test were used to compare clinical features, laboratory test results between groups.Results:Among the 18 patients in the MDA5-positive group, 7 were male and 11 were female, with an age of onset of 5.0 (2.6, 9.4) years and disease duration of 6.0 (4.0, 9.3) months. The MDA5-negative group included 36 cases (14 male, 22 female), with an age of onset of 4.9 (2.0, 7.0) years and disease duration of 5.0 (1.8, 7.0) months. The MDA5-positive group exhibited significantly higher rates of arthritis, skin ulcers, and interstitial lung disease (ILD), along with elevated serum ferritin (SF) and erythrocyte sedimentation rate levels compared to the MDA5-negative group (9/18 vs. 11% (4/36), 6/18 vs. 3% (1/36), 16/18 vs. 33% (12/36), 327 (141, 518) vs. 131 (68, 257) μg/L, 17.5 (12.5, 26.8) vs. 11.0 (5.0, 13.0) mm/1 h, χ2=7.92, 7.41, 14.84, Z=2.50, 2.87, all P<0.05). Conversely, the MDA5-positive group had lower rates of muscle weakness and lower creatine kinase levels (5/18 vs. 75% (27/36), 58.5 (49.3, 97.5) vs. 225.0 (68.0, 695.5) U/L, χ2=11.08, Z=-2.94, both P<0.05). Severe pulmonary involvement 6 cases and non-severe pulmonary involvement subgroups 12 cases. Among the MDA5-positive patients, those in the severe pulmonary involvement subgroup had an older age at onset and higher rates of muscle weakness as well as hydroxybutyrate dehydrogenase (HBDH), lactate dehydrogenase (LDH), SF, and Krebs von den Lungen-6 (KL-6) compared to the non-severe subgroup (all P<0.05). In the MDA5-positive group, 17 patients improved after treatment with glucocorticoids combined with immunosuppressants, while One died due to rapidly progressive ILD. Conclusions:Anti-MDA5 antibody-positive JDM is characterized by typical skin rashes, a high incidence of arthritis and skin ulcers, relatively mild muscle involvement, but is prone to ILD. Among MDA5-positive patients, those with older age at onset, muscle involvement (manifested as muscle weakness and elevated muscle enzymes (LDH, HBDH)), or significantly elevated KL-6 and SF levels are more likely to develop severe pulmonary complications.

Objective:To investigate the clinical features and prognosis of neuro-Beh?et′s syndrome (NBS) in children.Method:The clinical, brain magnetic resonance imaging and laboratory data of 5 children with NBS diagnosed in the Department of Pediatrics, General Hospital of Ningxia Medical University and Department of Rheumatology and Immunology, Children′s Hospital Affiliated to Capital Institute of Pediatrics from April 2014 to April 2024 were analyzed retrospectively. The follow-up method was retrospective outpatient or inpatient visit to evaluate the treatment effect of NBS.Result:Among the 5 NBS cases, 2 were male and 3 were female. The age of admission ranged from 8 to 17 years, the time from onset to diagnosis was 2 days to 4 years. Two patients had dizziness, headache and convulsions during the treatment of NBS, 1 patient had disturbance of consciousness, 1 patient gradually developed aphasia, limb movement disorder, dysphagia and muscle weakness after 4 years of Behcet's syndrome, and 1 patient had no clinical symptoms. C-reactive protein and erythrocyte sedimentation rate were increased in 4 cases, and cerebrospinal fluid white blood cells and immunoglobulin G were increased in 1 case. Brain magnetic resonance imaging of 4 children showed multiple lesions, including bilateral frontal lobe, occipital lobe, parietal lobe, periventricular and corpus callosum lesions. Brain magnetic resonance imaging showed multiple demyelinating diseases in 1 case, and cervical and thoracic magnetic resonance imaging showed slender cervical and thoracic spinal cord. All patients were treated with corticosteroids combined with immunosuppressants or biological agents. The children were followed up for 6 months to 4 years, and 4 cases had good treatment results, and 1 case finally gave up treatment.Conclusions:The clinical manifestations of NBS are not specific, and brain magnetic resonance imaging shows that the lesion location and morphology are not specific. NBS children treated with corticosteroids combined with immunosuppressive agents or biological agents have a good prognosis.